15 
Odontogenic Cysts and Tumors
Odontogenic cysts and tumors constitute an important
aspect of oral and maxillofacial pathology. Odontogenic
cysts are encountered relatively commonly in dental prac-
tice. Odontogenic tumors, by contrast, are uncommon
lesions. Even in the specialized oral and maxillofacial pathol-
ogy laboratory, less than 1% of all specimens received are
odontogenic tumors.
ODONTOGENIC CYSTS
With rare exceptions, epithelium-lined cysts in bone are
seen only in the jaws. Other than a few cysts that may result
from the inclusion of epithelium along embryonic lines of
fusion, most jaw cysts are lined by epithelium that is derived
from odontogenic epithelium. These are referred to as
odontogenic cysts. (Nonodontogenic jaw cysts are dis-
cussed in Chapter 1.)
Odontogenic cysts are subclassified as developmental or
inflammatory in origin. The inciting factors that initiate the
formation of developmental cysts are unknown, but these
lesions do not appear to be the result of an inflammatory
reaction. Inflammatory cysts are the result of inflamma-
tion. Box 15-1 presents categories of odontogenic cysts
modified from the 2005 World Health Organization
(WHO) classification. (The periapical cyst is discussed in
Chapter 3.)
◆ DENTIGEROUS CYST
(FOLLICULAR CYST)
The dentigerous cyst is defined as a cyst that originates by
the separation of the follicle from around the crown of an
unerupted tooth. This is the most common type of devel-
opmental odontogenic cyst, making up about 20% of all
epithelium-lined cysts of the jaws. The dentigerous cyst
encloses the crown of an unerupted tooth and is attached
to the tooth at the cementoenamel junction (Fig. 15-1). The
pathogenesis of this cyst is uncertain, but apparently it
develops by accumulation of fluid between the reduced
enamel epithelium and the tooth crown.
Although most dentigerous cysts are considered to be
developmental in origin, there are some examples that
appear to have an inflammatory pathogenesis. For example,
632
it has been suggested that, on occasion, a dentigerous cyst
may develop around the crown of an unerupted permanent
tooth as a result of periapical inflammation from an overly-
ing primary tooth. Another scenario involves a partially
erupted mandibular third molar that develops an inflamed
cystlike lesion along the distal or buccal aspect. Although
many such lesions probably are due to inflammation associ-
ated with recurrent pericoronitis, these lesions are usually
diagnosed as examples of dentigerous cyst, especially because
it is impossible to determine histopathologically whether
the inflammatory component is primary or secondary in
nature. The term paradental cyst sometimes has been
applied to these lesions, but the use of this term in the
literature is confusing because it also has been used to
describe examples of what is known as the buccal bifurcation
cyst (see page 650).
Clinical and Radiographic Features
Although dentigerous cysts may occur in association with
any unerupted tooth, most often they involve mandibular
third molars, accounting for approximately 65% of all cases.
Other relatively frequent sites include maxillary canines,
maxillary third molars, and mandibular second premolars.
Dentigerous cysts rarely involve unerupted deciduous teeth.
Occasionally, they are associated with supernumerary teeth
or odontomas. Multiple dentigerous cysts have been
reported, although this is an infrequent finding.
Although dentigerous cysts may be encountered in
patients across a wide age range, they are discovered most
frequently in patients between 10 and 30 years of age. There
is a slight male predilection, and the prevalence is higher
for whites than for blacks. Small dentigerous cysts are
usually completely asymptomatic and are discovered only
on a routine radiographic examination or when films are
taken to determine the reason for the failure of a tooth to
erupt. Dentigerous cysts can grow to a considerable size,
and large cysts may be associated with a painless expansion
of the bone in the involved area. Extensive lesions may
result in facial asymmetry. Large dentigerous cysts are
uncommon, and most lesions that are considered to be large
dentigerous cysts on radiographic examination prove to be
odontogenic keratocysts (OKCs) or ameloblastomas. Den-
tigerous cysts may become infected and be associated with
 CHAPTER 15  Odontogenic Cysts and Tumors 633

• BOX 15-1 Classification of Odontogenic Cysts

Developmental
• Dentigerous cyst
• Eruption cyst
• Odontogenic keratocyst (OKC)*
• Orthokeratinized odontogenic cyst
• Gingival (alveolar) cyst of the newborn
• Gingival cyst of the adult
• Lateral periodontal cyst
• Calcifying odontogenic cyst†
• Glandular odontogenic cyst

Inflammatory
• Periapical (radicular) cyst
• Residual periapical (radicular) cyst
• Fig. 15-2  Dentigerous Cyst. Central type showing the crown
• Buccal bifurcation cyst
projecting into the cystic cavity. (Courtesy of Dr. Stephen E. Irwin.)
*Although the OKC is included with the odontogenic tumors in the 2005 World
Health Organization (WHO) classification (“keratocystic odontogenic tumor”),
the authors still favor including it in the odontogenic cyst category.
†
Although the calcifying odontogenic cyst is included with odontogenic tumors
in the 2005 WHO classification (“calcifying cystic odontogenic tumor”), it is
discussed with the odontogenic cysts in this chapter.

• Fig. 15-3  Dentigerous Cyst. Lateral variety showing a large cyst

along the mesial root of the unerupted molar. This cyst exhibited
mucous cell prosoplasia. (Courtesy of Dr. John R. Cramer.)

• Fig. 15-1  Dentigerous Cyst. Gross specimen of a dentigerous
cyst involving a maxillary canine tooth. The cyst has been cut open to
show the cyst-to-crown relationship.
pain and swelling. Such infections may arise in a dentiger-
ous cyst that is associated with a partially erupted tooth or
by extension from a periapical or periodontal lesion that
affects an adjacent tooth.
Radiographically, the dentigerous cyst typically shows a
unilocular radiolucent area that is associated with the crown
of an unerupted tooth. The radiolucency usually has a well-
defined and often corticated border, but an infected cyst
may show ill-defined borders. A large dentigerous cyst may
give the impression of a multilocular process because of the
persistence of bone trabeculae within the radiolucency. The
cyst-to-crown relationship shows several radiographic varia-
tions. In the central variety, which is the most common,
the cyst surrounds the crown of the tooth and the crown
projects into the cyst (Fig. 15-2). The lateral variety is
usually associated with mesioangular impacted mandibular
third molars that are partially erupted. The cyst grows later-
ally along the root surface and partially surrounds the crown
(Fig. 15-3). In the circumferential variant, the cyst sur-
rounds the crown and extends for some distance along the
root so that a significant portion of the root appears to lie
within the cyst (Fig. 15-4). Rarely, a third molar may be
displaced to the lower border of the mandible or higher up
into the ascending ramus. Maxillary anterior teeth may be
displaced into the floor of the nose, and other maxillary
teeth may be moved through the maxillary sinus to the floor
of the orbit. Dentigerous cysts may displace the involved
tooth for a considerable distance. Root resorption of adja-
cent erupted teeth can occur.
Radiographic distinction between a small dentigerous
cyst and an enlarged follicle about the crown of an
unerupted tooth is difficult and may be largely an aca-
demic exercise (Fig. 15-5). For the lesion to be considered
a dentigerous cyst, some investigators believe that the
radiolucent space surrounding the tooth crown should
be at least 3 to 4  mm in diameter. Radiographic find-
ings are not diagnostic for a dentigerous cyst, however,
because OKCs, unilocular ameloblastomas, and many
other odontogenic and nonodontogenic tumors may have
634 C H A P T E R 1 5 Odontogenic Cysts and Tumors
• Fig. 15-4  Dentigerous Cyst. Circumferential variety showing cyst
extension along the mesial and distal roots of the unerupted tooth.
(Courtesy of Dr. Richard Marks.)
• Fig. 15-5  Dentigerous Cyst or Enlarged Follicle. Radiolucent
lesion involving the crown of an unerupted mandibular premolar. Dis-
tinction between a dentigerous cyst and an enlarged follicle for a lesion
of this size by radiographic and even histopathologic means is difficult,
if not impossible. (Courtesy of Dr. Wally Austelle.)
radiographic features that are essentially identical to those
of a dentigerous cyst.
Histopathologic Features
The histopathologic features of dentigerous cysts vary,
depending on whether the cyst is inflamed or not inflamed.
In the noninflamed dentigerous cyst, the fibrous connec-
tive tissue wall is loosely arranged and contains considerable
glycosaminoglycan ground substance. Small islands or cords
of inactive-appearing odontogenic epithelial rests may be
present in the fibrous wall. Occasionally these rests may be
numerous, and at times pathologists who are not familiar
with oral lesions have misinterpreted this finding as amelo-
blastoma. The epithelial lining consists of two to four layers
of flattened nonkeratinizing cells, and the epithelium and
connective tissue interface is flat (Fig. 15-6).
In the fairly common inflamed dentigerous cyst, the
fibrous wall is more collagenized, with a variable infiltration
of chronic inflammatory cells. The epithelial lining may
show varying amounts of hyperplasia with the development
• Fig. 15-6  Dentigerous Cyst. This noninflamed dentigerous cyst
shows a thin, nonkeratinized epithelial lining.
• Fig. 15-7  Dentigerous Cyst. This inflamed dentigerous cyst
shows a thicker epithelial lining with hyperplastic rete ridges. The
fibrous cyst capsule shows a diffuse chronic inflammatory infiltrate.
of rete ridges and more definite squamous features (Fig.
15-7). A keratinized surface is sometimes seen, but these
changes must be differentiated from those observed in the
OKC. Focal areas of mucous cells may be found in the
epithelial lining of dentigerous cysts (Fig. 15-8). Rarely,
ciliated columnar cells are present. Small nests of sebaceous
cells rarely may be noted within the fibrous cyst wall. These
mucous, ciliated, and sebaceous elements are believed to
represent the multipotentiality of the odontogenic epithelial
lining in a dentigerous cyst.
Gross examination of the wall of a dentigerous cyst may
reveal one or several areas of nodular thickening on the
luminal surface. These areas must be examined microscopi-
cally to rule out the presence of early neoplastic change.
Because a thin layer of reduced enamel epithelium nor-
mally lines the dental follicle surrounding the crown of an
unerupted tooth, it can be difficult to distinguish a small
dentigerous cyst from simply a normal or enlarged dental
follicle based on microscopic features alone. Again, this
distinction often represents largely an academic exercise; the
most important consideration is ensuring that the lesion
does not represent a more significant pathologic process
(e.g., OKC or ameloblastoma).
 CHAPTER 15  Odontogenic Cysts and Tumors 635

• Fig. 15-8  Dentigerous Cyst. Scattered mucous cells can be seen • Fig. 15-9  Eruption Cyst. This soft gingival swelling contains
within the epithelial lining. considerable blood and can also be designated as an eruption
hematoma.

Treatment and Prognosis

The usual treatment for a dentigerous cyst is careful enucle-
ation of the cyst together with removal of the unerupted
tooth. If eruption of the involved tooth is considered fea-
sible, then the tooth may be left in place after partial removal
of the cyst wall. Patients may need orthodontic treatment
to assist eruption. Large dentigerous cysts also may be
treated by marsupialization. This permits decompression of
the cyst, with a resulting reduction in the size of the bone
defect. The cyst can then be excised at a later date, with a
less extensive surgical procedure.
The prognosis for most dentigerous cysts is excellent, and
recurrence seldom is noted after complete removal of the
cyst. However, several potential complications must be con-
sidered. Much has been written about the possibility that • Fig. 15-10  Eruption Cyst. A cystic epithelial cavity can be seen
below the mucosal surface.
the lining of a dentigerous cyst might undergo neoplastic
transformation to an ameloblastoma. Although undoubt-
edly this can occur, the frequency of such neoplastic trans- associated with the deciduous mandibular central incisors,
formation is low. Rarely, a squamous cell carcinoma may the first permanent molars, and the deciduous maxillary
arise in the lining of a dentigerous cyst (see page 651). It is incisors. Surface trauma may result in a considerable amount
likely that some intraosseous mucoepidermoid carcino- of blood in the cystic fluid, which imparts a blue to purple-
mas (see page 457) develop from mucous cells in the lining brown color. Such lesions sometimes are referred to as erup-
of a dentigerous cyst. tion hematomas (Fig. 15-9).

◆ ERUPTION CYST
(ERUPTION HEMATOMA)
The eruption cyst is the soft tissue analogue of the dentiger-
ous cyst. The cyst develops as a result of separation of the
dental follicle from around the crown of an erupting tooth
that is within the soft tissues overlying the alveolar bone.
Clinical Features
The eruption cyst appears as a soft, often translucent swell-
ing in the gingival mucosa overlying the crown of an erupt-
ing deciduous or permanent tooth. Most examples are seen
in children younger than age 10. Although the cyst may
occur with any erupting tooth, the lesion is most commonly
Histopathologic Features
Intact eruption cysts seldom are submitted to the oral and
maxillofacial pathology laboratory, and most examples
consist of the excised roof of the cyst, which has been
removed to facilitate tooth eruption. These show surface
oral epithelium on the superior aspect. The underlying
lamina propria shows a variable inflammatory cell infiltrate.
The deep portion of the specimen, which represents the roof
of the cyst, shows a thin layer of nonkeratinizing squamous
epithelium (Fig. 15-10).
Treatment and Prognosis
Treatment may not be required because the cyst usually
ruptures spontaneously, permitting the tooth to erupt. If
636 C H A P T E R 1 5 Odontogenic Cysts and Tumors
this does not occur, then simple excision of the roof of the
cyst generally permits speedy eruption of the tooth.
◆ PRIMORDIAL CYST
The concept and meaning of the term primordial cyst
often have been controversial and confusing. In the older
classification of cysts used in the United States, the primor-
dial cyst was considered to originate from cystic degenera-
tion of the enamel organ epithelium before the development
of dental hard tissue. Therefore, the primordial cyst would
occur in place of a tooth.
In the mid-1950s, oral and maxillofacial pathologists in
Europe introduced the term odontogenic keratocyst
(OKC) to denote a cyst with specific histopathologic fea-
tures and clinical behavior, which was believed to arise from
the dental lamina (i.e., the dental primordium). Subse-
quently, this concept was widely accepted, and the terms
odontogenic keratocyst and primordial cyst were used synony-
mously. The 1972 WHO classification used the designation
primordial cyst as the preferred term for this lesion. The
1992 WHO classification, however, listed odontogenic kera-
tocyst as the preferred designation.
Almost all examples of so-called primordial cysts (i.e., a
cyst that develops in the place of a tooth) microscopically
will be OKCs (Fig. 15-11). Whether there could be such a
radiographic presentation that is not microscopically an
OKC is still unsettled. If such a lesion exists, then it must
be exceedingly rare.
◆ ODONTOGENIC KERATOCYST
(KERATOCYSTIC ODONTOGENIC TUMOR)
The odontogenic keratocyst (OKC) is a distinctive form
of developmental odontogenic cyst that deserves special
consideration because of its specific histopathologic
• Fig. 15-11  Primordial Cyst. This patient gave no history of extrac-
tion of the third molar. A cyst is located in the third molar area. The
cyst was excised, and histopathologic examination revealed an odon-
togenic keratocyst (OKC).
features and clinical behavior. There is general agreement
that the OKC arises from cell rests of the dental lamina.
This cyst shows a different growth mechanism and bio-
logic behavior from the more common dentigerous cyst
and radicular cyst. Most authors believe that dentigerous
and radicular cysts continue to enlarge as a result of
increased osmotic pressure within the lumen of the cyst.
This mechanism does not appear to hold true for OKCs,
and their growth may be related to genetic factors inher-
ent in the epithelium itself or enzymatic activity in the
fibrous wall.
Several investigators have suggested that the OKC be
regarded as a benign cystic neoplasm rather than a cyst, and
in the latest WHO monograph on head and neck tumors,
this lesion has been given the name keratocystic odonto-
genic tumor (KCOT). The arguments to support this
change in nomenclature largely rely on studies that have
shown certain molecular genetic alterations that are also
present in some neoplasms. When compared to other odon-
togenic cysts, the OKC shows significantly greater expres-
sion of proliferating cell nuclear antigen (PCNA) and
Ki-67, especially in the suprabasilar layer. Almost 30% of
sporadic OKCs and over 85% of OKCs associated with the
nevoid basal cell carcinoma syndrome show mutations of
PTCH1, an important molecule in the Hedgehog signalling
pathway. Also, genetic analyses have demonstrated loss of
heterozygosity for various other tumor suppressor genes
(p16, p53, MCC, TSLC1, LATS2, and FHIT) in many
OKCs.
Whether such molecular findings warrant reclassification
of the OKC as a neoplasm (KCOT) remains a hotly debated
topic in oral pathology circles. The authors currently favor
retaining “odontogenic keratocyst” as the primary term for
this lesion, although both terms will be found in the litera-
ture and should be considered synonymous. Regardless of
which term is preferred, these lesions are significant for
three reasons:
1. Greater growth potential than most other odontogenic
cysts
2. Higher recurrence rate
3. Possible association with the nevoid basal cell carcinoma
syndrome
Although there are wide variations in the reported fre-
quency of OKCs compared with that of other types of
odontogenic cysts, most studies indicate that OKCs make
up 3% to 11% of all odontogenic cysts.
Clinical and Radiographic Features
OKCs may be found in patients who range in age from
infancy to old age, but about 60% of all cases are diagnosed
in people between 10 and 40 years of age. There is a slight
male predilection. The mandible is involved in 60% to 80%
of cases, with a marked tendency to involve the posterior
body and ramus (Fig. 15-12).
Small OKCs are usually asymptomatic and discovered
only during the course of a radiographic examination.

20% 2% 13%
49% 7% 9%
• Fig. 15-12  Odontogenic Keratocyst (OKC). Relative distribution
of OKCs in the jaws.
• Fig. 15-13  Odontogenic Keratocyst (OKC). Large, multilocular
cyst involving most of the ascending ramus. (Courtesy of Dr. S.C.
Roddy.)
Larger OKCs may be associated with pain, swelling, or
drainage. Some extremely large cysts, however, may cause
no symptoms.
OKCs tend to grow in an anteroposterior direction
within the medullary cavity of the bone without causing
obvious bone expansion. This feature may be useful in dif-
ferential clinical and radiographic diagnosis because dentig-
erous and radicular cysts of comparable size are usually
associated with bony expansion. Multiple OKCs may be
present, and such patients should be evaluated for other
manifestations of the nevoid basal cell carcinoma (Gorlin)
syndrome (see page 640).
OKCs demonstrate a well-defined radiolucent area with
smooth and often corticated margins. Large lesions, par-
ticularly in the posterior body and ramus of the mandible,
may appear multilocular (Fig. 15-13). An unerupted tooth
is involved in the lesion in 25% to 40% of cases; in such
instances, the radiographic features suggest the diagnosis of
dentigerous cyst (Figs. 15-14 and 15-15). In these cases, the
cyst has presumably arisen from dental lamina rests near an
unerupted tooth and has grown to envelop the unerupted
tooth. Resorption of the roots of erupted teeth adjacent to
CHAPTER 15  Odontogenic Cysts and Tumors 637
• Fig. 15-14  Odontogenic Keratocyst (OKC). This cyst involves
the crown of an unerupted premolar. Radiographically, this lesion
cannot be differentiated from a dentigerous cyst.
• Fig. 15-15  Odontogenic Keratocyst (OKC). Computed tomog-
raphy (CT) scan showing a large cyst involving the crown of an
unerupted maxillary third molar. The cyst largely fills the maxillary sinus.
(Courtesy of Dr. E.B. Bass.)
OKCs is less common than that noted with dentigerous and
radicular cysts.
The diagnosis of OKC is based on the histopathologic
features. The radiographic findings, although often highly
suggestive, are not diagnostic. The radiographic findings in
an OKC may simulate those of a dentigerous cyst, a radicu-
lar cyst, a residual cyst, a lateral periodontal cyst (Fig.
15-16), or the so-called globulomaxillary cyst (which is no
longer considered to be a true entity). OKCs of the anterior
midline maxillary region can mimic nasopalatine duct cysts.
For unknown reasons, this particular subset of keratocyst
usually occurs in older individuals with a mean age of nearly
70 years. Rare examples of peripheral OKCs within the
gingival soft tissues have been reported.
638 C H A P T E R 1 5 Odontogenic Cysts and Tumors
• Fig. 15-16  Odontogenic Keratocyst (OKC). This cyst cannot be
radiographically differentiated from a lateral periodontal cyst. (Courtesy
of Dr. Keith Lemmerman.)
Histopathologic Features
The OKC typically shows a thin, friable wall, which is
often difficult to enucleate from the bone in one piece.
The cystic lumen may contain a clear liquid that is similar
to a transudate of serum, or it may be filled with a cheesy
material that, on microscopic examination, consists of
keratinaceous debris. Microscopically, the thin fibrous wall
is usually devoid of significant inflammation. The epithelial
lining is composed of a uniform layer of stratified squa-
mous epithelium, usually six to eight cells in thickness.
The epithelium and connective tissue interface is usually
flat, and rete ridge formation is inconspicuous. Detachment
of portions of the cyst-lining epithelium from the fibrous
wall is commonly observed. The luminal surface shows
flattened parakeratotic epithelial cells, which exhibit a wavy
or corrugated appearance (Fig. 15-17). On occasion, iso-
lated foci of orthokeratin production may be found in
addition to the parakeratin. The basal epithelial layer is
composed of a palisaded layer of cuboidal or columnar
epithelial cells, which are often hyperchromatic. Small
satellite cysts, cords, or islands of odontogenic epithelium
may be seen within the fibrous wall. These structures have
been present in 7% to 26% of cases in various reported
series. In rare instances, cartilage has been observed in the
wall of an OKC.
In the presence of inflammatory changes, the typical
features of the OKC may be altered. The parakeratinized
luminal surface may disappear, and the epithelium may
proliferate to form rete ridges with the loss of the charac-
teristic palisaded basal layer (Fig. 15-18). When these
changes involve most of the cyst lining, the diagnosis of
• Fig. 15-17  Odontogenic Keratocyst (OKC). The epithelial lining
is 6 to 8 cells thick, with a hyperchromatic and palisaded basal cell
layer. Note the corrugated parakeratotic surface.
• Fig. 15-18  Odontogenic Keratocyst (OKC). The characteristic
microscopic features have been lost in the central area of this portion
of the cystic lining because of the heavy chronic inflammatory cell
infiltrate.
OKC cannot be confirmed unless other sections show the
typical features described earlier.
In the past, some investigators recognized a purely ortho-
keratotic variant of the OKC. However, these cysts do not
demonstrate a hyperchromatic and palisaded basal cell layer,
which is so characteristic of true OKCs. In addition, the
clinical behavior of these orthokeratinized cysts differs
markedly from that of the typical parakeratinized cysts
described in this section. The authors believe that it is more
logical to discuss these orthokeratinizing cysts separately
(see following section).
Treatment and Prognosis
Although the presence of an OKC may be suspected on
clinical or radiographic grounds, histopathologic confirma-
tion is required for the diagnosis. Consequently, most
OKCs are treated similarly to other odontogenic cysts—i.e.,
by enucleation and curettage. Complete removal of the cyst
in one piece is often difficult because of the thin, friable
nature of the cyst wall. In contrast to other odontogenic
 CHAPTER 15  Odontogenic Cysts and Tumors 639

cysts, OKCs often tend to recur after treatment. Whether

this is due to fragments of the original cyst that were not
removed at the time of the operation or a “new” cyst that
has developed from dental lamina rests in the general area
of the original cyst cannot be determined with certainty.
The reported frequency of recurrence in various studies
ranges from 5% to 62%. This wide variation may be related
to the total number of cases studied, the length of follow-up
periods, and the inclusion or exclusion of orthokeratinized
cysts in the study group. Several reports that include large
numbers of cases indicate a recurrence rate of approximately
30%. Recurrence is encountered more often in mandibular
OKCs, particularly those in the posterior body and ramus. A
Multiple recurrences are not unusual. Although many
OKCs recur within 5 years of the original surgery, a signifi-
cant number of recurrences may not be manifested until 10
or more years after the original surgical procedure. Long-
term clinical and radiographic follow-up, therefore, is
necessary.
Many surgeons recommend peripheral ostectomy of the
bony cavity with a bone bur to reduce the frequency of
recurrence. Others advocate chemical cauterization of the
bony cavity with Carnoy’s solution after cyst removal
(although use of Carnoy’s solution may not be permitted
by some hospitals). Intraluminal injection of Carnoy’s solu-
tion also has been used to free the cyst from the bony B
wall, thereby allowing easier removal with a lower recur-
rence rate. After cystotomy and incisional biopsy, some • Fig. 15-19  Decompression of an Odontogenic Keratocyst
(OKC). A, Large unilocular radiolucency associated with the right
surgeons have treated large OKCs by insertion of a poly- mandibular third molar. B, Six months after insertion of a polyethylene
ethylene drainage tube to allow decompression and sub- drainage tube to allow decompression, the cyst has shrunk and the
sequent reduction in size of the cystic cavity (Fig. 15-19). third molar has migrated downward and forward. (Courtesy of Dr. Tom
Such decompression treatment results in thickening of the Szakal.)
cyst lining, allowing easier removal with an apparently
lower recurrence rate. odontogenic keratocyst, it is generally accepted that they are
Other than the tendency for recurrences, the overall clinicopathologically different from the more common
prognosis for most OKCs is good. Occasionally, a locally parakeratinized odontogenic keratocyst (OKC) and should
aggressive OKC cannot be controlled without local resec- be placed into a different category. Orthokeratinized odon-
tion and bone grafting. In extremely rare instances, kerato- togenic cysts represent 7% to 17% of all keratinizing jaw
cysts have been seen to extend up into the skull base region. cysts.
A few examples of carcinoma arising in an OKC have been
reported, but the propensity for an OKC to undergo malig- Clinical and Radiographic Features
nant alteration is no greater and is possibly less than that
for other types of odontogenic cysts. Patients with OKCs Orthokeratinized odontogenic cysts occur predominantly
should be evaluated for manifestations of the nevoid basal in young adults and show over a 2 : 1 male-to-female ratio.
cell carcinoma syndrome (see page 640), particularly if the The lesion occurs more frequently in the mandible than the
patient is in the first or second decade of life or if multiple maxilla (3 : 1 ratio), with a tendency to involve the posterior
keratocysts are identified. areas of the jaws. They have no clinical or radiographic
features that differentiate them from other inflammatory or
developmental odontogenic cysts. The lesion usually appears
◆ORTHOKERATINIZED as a unilocular radiolucency, but occasional examples have
ODONTOGENIC CYST been multilocular. About two-thirds of orthokeratinized
odontogenic cysts are encountered in a lesion that appears
The designation orthokeratinized odontogenic cyst does clinically and radiographically to represent a dentigerous
not denote a specific clinical type of odontogenic cyst but cyst; they most often involve an unerupted mandibular
refers only to an odontogenic cyst that microscopically has third molar tooth (Figs. 15-20 and 15-21). The size can vary
an orthokeratinized epithelial lining. Although such lesions from less than 1 cm to large lesions greater than 7 cm in
were originally called the orthokeratinized variant of diameter.
640 C H A P T E R 1 5 Odontogenic Cysts and Tumors
• Fig. 15-20  Orthokeratinized Odontogenic Cyst. Small unilocu-
lar radiolucency associated with the impacted mandibular left third
molar. (Courtesy of Dr. Tom McDonald.)
• Fig. 15-21  Orthokeratinized Odontogenic Cyst. A large cyst
involving a horizontally impacted lower third molar. (Courtesy of 
Dr. Carroll Gallagher.)
Histopathologic Features
The cyst lining is composed of stratified squamous epithe-
lium, which shows an orthokeratotic surface of varying
thickness. Keratohyaline granules may be prominent in the
superficial epithelial layer subjacent to the orthokeratin. The
epithelial lining may be relatively thin, and a prominent
palisaded basal layer, characteristic of the OKC, is not
present (Fig. 15-22).
Treatment and Prognosis
Enucleation with curettage is the usual treatment for ortho-
keratinized odontogenic cysts. Recurrence has rarely been
noted, and the reported frequency is around 2%, which is
in marked contrast with the 30% or higher recurrence rate
associated with OKCs. It has been suggested that cysts with
an orthokeratinized surface may be at slightly greater risk
for malignant transformation, but evidence for this is scant.
Orthokeratinized odontogenic cysts typically are not associ-
ated with nevoid basal cell carcinoma syndrome.
• Fig. 15-22  Orthokeratinized Odontogenic Cyst. Microscopic
features showing a thin epithelial lining. The basal epithelial layer does
not demonstrate palisading. Keratohyaline granules are present, and
a thick layer of orthokeratin is seen on the luminal surface.
◆NEVOID BASAL CELL CARCINOMA
SYNDROME (GORLIN SYNDROME)
Nevoid basal cell carcinoma syndrome (Gorlin syn-
drome) is an autosomal dominant inherited condition that
exhibits high penetrance and variable expressivity. The syn-
drome is caused by mutations in patched (PTCH), a tumor
suppressor gene that has been mapped to chromosome
9q22.3-q31. Approximately 35% to 50% of affected
patients represent new mutations. One of the most common
clinical features is development of OKCs, which can lead
to early diagnosis. The prevalence of Gorlin syndrome is
estimated to be anywhere from 1 in 19,000 to 1 in 256,000,
depending on the population studied.
Clinical and Radiographic Features
There is great variability in the expressivity of nevoid basal
cell carcinoma syndrome, and no single component is
present in all patients. The most common and significant
features are summarized in Box 15-2. The patient often has
a characteristic facies, with frontal and temporoparietal
bossing, which results in an increased cranial circumference
(more than 60 cm in adults). The eyes may appear widely
separated, and many patients have true mild ocular hyper-
telorism. Mild mandibular prognathism is also commonly
present (Fig. 15-23).
Basal cell carcinomas of the skin are a major component
of the syndrome. These tumors usually begin to appear at
puberty or in the second and third decades of life, although
they can develop in young children. The lesions may vary
from flesh-colored papules to ulcerating plaques. They often
appear on skin that is not exposed to sunlight, but they are
most commonly located in the midface area (Fig. 15-24).
The number of skin tumors may vary from only a few to
many hundreds. Blacks with the syndrome tend to develop
basal cell carcinomas less frequently than whites (40%
versus 90%), and they have fewer of these lesions, probably
because of protective skin pigmentation.
 CHAPTER 15  Odontogenic Cysts and Tumors 641

• BOX 15-2 Major Clinical Features of the Nevoid

Basal Cell Carcinoma Syndrome
50% or Greater Frequency
• Multiple basal cell carcinomas
• Odontogenic keratocysts (OKCs)
• Epidermal cysts of the skin
• Palmar/plantar pits
• Calcified falx cerebri
• Enlarged head circumference
• Rib anomalies (splayed, fused, partially missing, and/or bifid)
• Mild ocular hypertelorism
• Spina bifida occulta of cervical or thoracic vertebrae

15% to 49% Frequency

• Calcified ovarian fibromas
• Short fourth metacarpals
• Kyphoscoliosis or other vertebral anomalies
• Pectus excavatum or carinatum
• Strabismus (exotropia)

Less than 15% Frequency (but Not Random)

• Medulloblastoma
• Meningioma
• Lymphomesenteric cysts
• Cardiac fibroma
• Fetal rhabdomyoma
• Marfanoid build
• Cleft lip and/or palate • Fig. 15-23  Nevoid Basal Cell Carcinoma Syndrome. This
• Hypogonadism in males 11-year-old girl shows hypertelorism and mandibular swelling. (Cour-
• Intellectual disability tesy of Dr. Richard DeChamplain.)
From Gorlin RJ: Nevoid basal-cell carcinoma syndrome, Medicine 66:98-113,
1987.

Palmar and plantar pits are present in about 65% to 85%

of patients (Fig. 15-25). These punctate lesions represent a
localized impairment of the maturation of basal epithelial
cells, resulting in a focally depressed area as the result of a
markedly thinned keratin layer. Basal cell carcinomas rarely
may develop at the base of the pits.
Ovarian cysts and fibromas have been reported in 25%
to 50% of women with this syndrome. A number of other
tumors also have been reported to occur with lesser fre-
quency. These include medulloblastoma within the first 2
years of life, meningioma, cardiac fibroma, and fetal
rhabdomyoma. • Fig. 15-24  Nevoid Basal Cell Carcinoma Syndrome. An ulcer-
Skeletal anomalies are present in 60% to 75% of patients ating basal cell carcinoma is present on the upper face.
with this syndrome. The most common anomaly is a bifid
rib or splayed ribs (Fig. 15-26). This anomaly may involve
several ribs and may be bilateral. Kyphoscoliosis has been are OKCs, although there are some differences between the
observed in about 30% to 40% of patients, and a number cysts in patients with nevoid basal cell carcinoma syndrome
of other anomalies, such as spina bifida occulta and short- and in those with isolated OKCs. The cysts are frequently
ened metacarpals, seem to occur with unusual frequency. A multiple; some patients have had as many as ten separate
distinctive lamellar calcification of the falx cerebri, noted on cysts. The patient’s age when the first OKC is removed is
an anteroposterior skull radiograph or computed tomogra- significantly younger in those affected by this syndrome
phy (CT) image, is a common finding and is present in than in those with isolated OKCs. For most patients with
most affected patients (Fig. 15-27). this syndrome, their first OKC is removed before age 19.
Jaw cysts are one of the most constant features of the About one-third of patients with nevoid basal cell carci-
syndrome and are present in 90% of the patients. The cysts noma syndrome have only a solitary cyst at the time of the
642 C H A P T E R 1 5 Odontogenic Cysts and Tumors
• Fig. 15-25  Nevoid Basal Cell Carcinoma Syndrome. Plantar
pits.
• Fig. 15-26  Nevoid Basal Cell Carcinoma Syndrome. Chest film
showing presence of bifid ribs (arrows).
initial presentation, but in most cases additional cysts will
develop over periods ranging from 1 to 20 years.
Radiographically, the cysts in patients with nevoid basal
cell carcinoma syndrome do not differ significantly from
isolated OKCs. The cysts in patients with this syndrome are
often associated with the crowns of unerupted teeth; on
radiographs they may mimic dentigerous cysts (Fig. 15-28).
Diagnostic criteria for the nevoid basal cell carcinoma
syndrome are provided in Box 15-3.
Histopathologic Features
The cysts in the nevoid basal cell carcinoma syndrome
histopathologically are invariably OKCs. The keratocysts
in patients with this syndrome tend to have more sat­
ellite cysts, solid islands of epithelial proliferation, and
• Fig. 15-27  Nevoid Basal Cell Carcinoma Syndrome. Antero-
posterior skull film showing calcification of the falx cerebri. (Courtesy
of Dr. Ramesh Narang.)
odontogenic epithelial rests within the fibrous capsule
than do isolated keratocysts (Fig. 15-29). Foci of calci-
fication also appear to be more common. These features,
however, are not diagnostic for nevoid basal cell carcinoma
syndrome because they may be seen in isolated kerato-
cysts. OKCs associated with this syndrome have been
shown to demonstrate overexpression of p53 and cyclin
D1 (bcl-1) oncoproteins when compared with nonsyn-
drome keratocysts.
The basal cell tumors of the skin cannot be distinguished
from ordinary basal cell carcinomas. They exhibit a wide
spectrum of histopathologic findings, from superficial
basal cell lesions to aggressive, noduloulcerative basal cell
carcinomas.
Treatment and Prognosis
Most of the anomalies in nevoid basal cell carcinoma syn-
drome are minor and usually not life threatening. The
prognosis generally depends on the behavior of the skin
tumors. In a few cases, aggressive basal cell carcinomas
have caused the death of the patient as a result of tumor
invasion of the brain or other vital structures (Figs. 15-30
and 15-31). Because the development of the basal cell
carcinomas seems to be triggered by ultraviolet (UV) light
exposure, patients should take appropriate precautions to
avoid sunlight. For the same reason, radiation therapy
should be avoided if at all possible. The jaw cysts are
treated in the same manner as isolated OKCs, but in many
patients additional cysts will continue to develop. Varying
degrees of jaw deformity may result from the operations
for multiple cysts. Infection of the cysts in patients with
 CHAPTER 15  Odontogenic Cysts and Tumors 643

• BOX 15-3 Diagnostic Criteria for the Nevoid Basal Cell Carcinoma Syndrome
A diagnosis can be made if the patient has: Minor Criteria
1. Two major criteria 1. Macrocephaly
2. One major and two minor criteria 2. Congenital malformation: Cleft lip or palate, frontal bossing,
3. One major criterion and genetic confirmation coarse facial features, and/or hypertelorism
3. Preaxial or postaxial polydactyly
Major Criteria 4. Rib or vertebral abnormalities: bifid, splayed, or extra ribs;
1. Five or more basal cell carcinomas or one before the age of bifid vertebrae
30 years 5. Ovarian or cardiac fibromas
2. Odontogenic keratocyst (OKC) 6. Medulloblastoma*
3. Lamellar calcification of the falx cerebri 7. Ocular anomalies: Cataract, coloboma, and/or
4. Two or more palmar or plantar pits microphthalmia
5. First degree relative with the nevoid basal cell carcinoma 8. Lymphomesenteric or pleural cysts
syndrome
*In a recent consensus conference, it was suggested that medulloblastoma should be considered a major criterion.

• Fig. 15-28  Nevoid Basal Cell Carcinoma Syndrome. Large cysts are present in the right and left
mandibular molar regions, together with a smaller cyst involving the right maxillary canine in the same
patient shown in Fig. 15-23. (Courtesy of Dr. Richard DeChamplain.)

• Fig. 15-29  Nevoid Basal Cell Carcinoma Syndrome. Odonto-
genic keratocyst (OKC) showing numerous odontogenic epithelial rests
in the cyst wall.
• Fig. 15-30  Nevoid Basal Cell Carcinoma Syndrome. This
52-year-old man had more than 100 basal cell carcinomas removed
from his face over a 30-year period. Several basal cell carcinomas are
present in this photograph. The lesion at the inner canthus of the left
eye was deeply invasive and was eventually fatal as a result of brain
invasion.
644 C H A P T E R 1 5 Odontogenic Cysts and Tumors
• Fig. 15-31  Nevoid Basal Cell Carcinoma Syndrome. Facial
deformity secondary to multiple surgical procedures to remove basal
cell carcinomas.
this syndrome is also relatively common. Some investigators
have suggested that affected children should have magnetic
resonance imaging (MRI) studies every 6 months until 7
years of age to monitor for the development of medullo-
blastoma. Genetic counseling is appropriate for affected
individuals.
◆GINGIVAL (ALVEOLAR) CYST
OF THE NEWBORN
Gingival cysts of the newborn are small, superficial,
keratin-filled cysts that are found on the alveolar mucosa of
infants. These cysts arise from remnants of the dental
lamina. They are common lesions, having been reported in
up to half of all newborns. However, because they disappear
spontaneously by rupture into the oral cavity, the lesions
seldom are noticed or sampled for biopsy. Similar inclusion
cysts (e.g., Epstein’s pearls and Bohn’s nodules) are also
found in the midline of the palate or laterally on the hard
and soft palate (see page 24).
Clinical Features
Gingival cysts of the newborn appear as small, usually mul-
tiple whitish papules on the mucosa overlying the alveolar
processes of neonates (Fig. 15-32). The individual cysts are
• Fig. 15-32  Gingival Cyst of the Newborn. Multiple whitish
papules on the alveolar ridge of a newborn infant.
usually no more than 2 to 3 mm in diameter. The maxillary
alveolus is more commonly involved than the mandibular.
Histopathologic Features
Examination of an intact gingival cyst of the newborn
shows a thin, flattened epithelial lining with a parakeratotic
luminal surface. The lumen contains keratinaceous debris.
Treatment and Prognosis
No treatment is indicated for gingival cysts of the newborn
because the lesions spontaneously involute as a result of the
rupture of the cysts and resultant contact with the oral
mucosal surface. The lesions are rarely seen after 3 months
of age.
◆ GINGIVAL CYST OF THE ADULT
The gingival cyst of the adult is an uncommon lesion. It
is considered to represent the soft tissue counterpart of the
lateral periodontal cyst (see next topic), being derived
from rests of the dental lamina (rests of Serres). The diag-
nosis of gingival cyst of the adult should be restricted to
lesions with the same histopathologic features as those of
the lateral periodontal cyst. On rare occasions, a cyst may
develop in the gingiva at the site of a gingival graft; however,
such lesions probably represent epithelial inclusion cysts that
are a result of the surgical procedure.
Clinical Features
Like the lateral periodontal cyst, the gingival cyst of the
adult shows a striking predilection to occur in the mandibu-
lar canine and premolar area (60% to 75% of cases). Gin-
gival cysts of the adult are most commonly found in patients
in the fifth and sixth decades of life. They are almost invari-
ably located on the facial gingiva or alveolar mucosa. Maxil-
lary gingival cysts are usually found in the incisor, canine,
and premolar areas.

• Fig. 15-33  Gingival Cyst of the Adult. Tense, fluid-filled swelling
on the facial gingiva.
• Fig. 15-34  Gingival Cyst of the Adult. Low-power photomicro-
graph showing a thin-walled cyst in the gingival soft tissue.
Clinically, the cysts appear as painless, domelike swell-
ings, usually less than 0.5 cm in diameter, although rarely
they may be somewhat larger (Fig. 15-33). They are often
bluish or blue-gray. In some instances, the cyst may cause
a superficial “cupping out” of the alveolar bone, which is
usually not detected on a radiograph but is apparent when
the cyst is excised. If more bone is missing, one could argue
that the lesion may be a lateral periodontal cyst that has
eroded the cortical bone rather than a gingival cyst that
originated in the mucosa.
Histopathologic Features
The histopathologic features of the gingival cyst of the adult
are similar to those of the lateral periodontal cyst, consisting
of a thin, flattened epithelial lining with or without focal
plaques that contain clear cells (Figs. 15-34 and 15-35).
Small nests of these glycogen-rich clear cells, which repre-
sent rests of the dental lamina, also may be seen in the
surrounding connective tissue. Sometimes the cystic lining
is so thin that it is easily mistaken for the endothelial lining
of a dilated blood vessel.
CHAPTER 15  Odontogenic Cysts and Tumors 645
• Fig. 15-35  Gingival Cyst of the Adult. High-power photomicro-
graph showing a plaquelike thickening of the epithelial lining.
Treatment and Prognosis
The gingival cyst of the adult responds well to simple surgi-
cal excision. The prognosis is excellent.
◆ LATERAL PERIODONTAL CYST
(BOTRYOID ODONTOGENIC CYST)
The lateral periodontal cyst is an uncommon type of
developmental odontogenic cyst that typically occurs along
the lateral root surface of a tooth. It is believed to arise from
rests of the dental lamina, and it represents the intrabony
counterpart of the gingival cyst of the adult. The lateral
periodontal cyst accounts for less than 2% of all epithelium-
lined jaw cysts.
In the past, the term lateral periodontal cyst was used to
describe any cyst that developed along the lateral root
surface, including lateral radicular cysts (see page 120) and
OKCs (see page 636). However, the lateral periodontal cyst
has distinctive clinical and microscopic features that distin-
guish it from other lesions that sometimes develop in the
same location.
Clinical and Radiographic Features
The lateral periodontal cyst is most often an asymptomatic
lesion that is detected only during a radiographic examina-
tion. It most frequently occurs in patients in the fifth
through the seventh decades of life; rarely does it occur in
someone younger than age 30. Around 75% to 80% of
cases occur in the mandibular premolar-canine-lateral
incisor area. Maxillary examples also usually involve this
same tooth region (Fig. 15-36).
Radiographically, the cyst usually appears as a well-
circumscribed radiolucent area located laterally to the root
or roots of vital teeth. Most such cysts are less than 1.0 cm
in greatest diameter (Figs. 15-37 and 15-38). In rare
instances, multifocal lateral periodontal cysts have been
described. In addition, some examples can develop in eden-
tulous sites.
646 C H A P T E R 1 5 Odontogenic Cysts and Tumors
1% 15% 4%
1% 66% 14%
• Fig. 15-36  Lateral Periodontal Cyst. Relative distribution of
lateral periodontal cysts in the jaws.
• Fig. 15-37  Lateral Periodontal Cyst. Radiolucent lesion between
the roots of a vital mandibular canine and first premolar.
Occasionally, the lesion may have a polycystic appear-
ance; such examples have been termed botryoid odonto-
genic cysts. Grossly and microscopically, they show a
grapelike cluster of small individual cysts (Fig. 15-39).
These lesions are generally considered to represent a variant
of the lateral periodontal cyst, possibly the result of cystic
degeneration and subsequent fusion of adjacent foci of
dental lamina rests. The botryoid variant often shows a
multilocular radiographic appearance, but it also may
appear unilocular.
The radiographic features of the lateral periodontal cyst
are not diagnostic; an OKC that develops between the roots
of adjacent teeth may show identical radiographic findings.
An inflammatory radicular cyst that occurs laterally to a
root in relation to an accessory foramen or a cyst that arises
from periodontal inflammation also may simulate a lateral
• Fig. 15-38  Lateral Periodontal Cyst. A larger lesion causing root
divergence.
• Fig. 15-39  Lateral Periodontal Cyst. Gross specimen of a bot-
ryoid variant. Microscopically, this grapelike cluster revealed three
separate cavities.
periodontal cyst radiographically (see page 120). In one
study of 46 cases of cystic lesions in the lateral periodontal
region, only 13 met the histopathologic criteria for the
lateral periodontal cyst; eight were OKCs, 20 were inflam-
matory cysts, and five were of undetermined origin.
Histopathologic Features
The lateral periodontal cyst has a thin, generally nonin-
flamed, fibrous wall, with an epithelial lining that is only
one to three cells thick in most areas. This epithelium
usually consists of flattened squamous cells, but sometimes
the cells are cuboidal in shape. Foci of glycogen-rich clear
cells may be interspersed among the lining epithelial cells.
Some cysts show focal nodular thickenings of the lining

B 1. Calcifying cystic odontogenic tumor
• Fig. 15-40  Lateral Periodontal Cyst. A, This photomicrograph
shows a thin epithelial lining with focal nodular thickenings. B, These
thickenings often show a swirling appearance of the cells.
epithelium, which are composed chiefly of clear cells (Fig.
15-40). Clear cell epithelial rests sometimes are seen within
the fibrous wall. Rarely, lateral periodontal cysts exhibit
focal areas that histopathologically are suggestive of the
glandular odontogenic cyst (see page 649).
Treatment and Prognosis
Conservative enucleation of the lateral periodontal cyst is
the treatment of choice. Usually, this can be accomplished
without damage to the adjacent teeth. Recurrence is unusual,
although it has been reported with the botryoid variant,
presumably because of its polycystic nature. An exceedingly
rare case of squamous cell carcinoma, which apparently
originated in a lateral periodontal cyst, also has been
reported.
◆ CALCIFYING ODONTOGENIC CYST
(CALCIFYING CYSTIC ODONTOGENIC
TUMOR; GORLIN CYST; DENTINOGENIC
GHOST CELL TUMOR; GHOST CELL
ODONTOGENIC CARCINOMA)
First described in 1962 by Gorlin and associates, the calci-
fying odontogenic cyst is part of a spectrum of lesions
CHAPTER 15  Odontogenic Cysts and Tumors 647
2% 12% 38%
11% 10% 27%
• Fig. 15-41  Calcifying Odontogenic Cyst. Relative distribution of
calcifying odontogenic cysts in the jaws.
characterized by odontogenic epithelium containing “ghost
cells,” which then may undergo calcification. Although
most examples grow in a cystic fashion, some lesions
occur as solid tumorlike growths (dentinogenic ghost cell
tumor). Therefore, in the current WHO classification
system, these lesions have been categorized as odontogenic
tumors under three categories (based on the cystic, solid, or
malignant nature of the lesion):
2. Dentinogenic ghost cell tumor
3. Ghost cell odontogenic carcinoma
The overwhelming majority of intraosseous ghost cell
odontogenic lesions grow as cystic lesions, and less than 5%
of cases can be classified as solid dentinogenic ghost cell
tumors. Therefore, the authors still prefer to use the simpler
designation “calcifying odontogenic cyst” for the cystic
examples. Approximately one-third of peripheral lesions
will be solid in nature, although these peripheral examples
are not as aggressive as their intraosseous counterparts.
The calcifying odontogenic cyst may be associated with
other recognized odontogenic tumors, most commonly
odontomas. However, adenomatoid odontogenic tumors
and ameloblastomas have also been associated with calcify-
ing odontogenic cysts.
Clinical and Radiographic Features
Intraosseous calcifying odontogenic cysts occur with about
equal frequency in the maxilla and mandible. About 65%
of cases are found in the incisor and canine areas (Fig.
15-41). The mean age is 30 years, and most cases are diag-
nosed in the second to fourth decades of life. Calcifying
odontogenic cysts that are associated with odontomas tend
to occur in younger patients, with a mean age of 17 years.
The central calcifying odontogenic cyst is usually a uni-
locular, well-defined radiolucency, although the lesion occa-
sionally may appear multilocular. Radiopaque structures
within the lesion, either irregular calcifications or toothlike
densities, are present in about one-third to one-half of cases
(Fig. 15-42). In approximately one-third of cases, the radio-
lucent lesion is associated with an unerupted tooth, most
648 C H A P T E R 1 5 Odontogenic Cysts and Tumors
often a canine. Most calcifying odontogenic cysts are
between 2.0 and 4.0 cm in greatest diameter, but lesions as
large as 12.0 cm have been noted. Root resorption or diver-
gence of adjacent teeth is seen with some frequency (Fig.
15-43).
Extraosseous examples comprise from 5% to 17% of all
cases, appearing as localized sessile or pedunculated gingival
masses with no distinctive clinical features (Fig. 15-44).
They can resemble common gingival fibromas, gingival
cysts, or peripheral giant cell granulomas. Peripheral exam-
ples tend to occur later in life, with peak prevalence during
the sixth to eighth decades.
Histopathologic Features
The calcifying odontogenic cyst most commonly occurs as
a well-defined cystic lesion with a fibrous capsule and a
lining of odontogenic epithelium of four to ten cells in
thickness. The basal cells of the epithelial lining may be
cuboidal or columnar and are similar to ameloblasts. The
• Fig. 15-42  Calcifying Odontogenic Cyst. Well-circumscribed
mixed radiolucent/radiopaque lesion in the right body of the mandible.
(Courtesy of Dr. John Wright.)
A B
• Fig. 15-43  Calcifying Odontogenic Cyst. A, Expansion of the posterior maxillary alveolus caused
by a large calcifying odontogenic cyst. B, Panoramic radiograph of the same patient showing a large
radiolucency in the posterior maxilla. A small calcified structure is seen in the lower portion of the cyst.
(Courtesy of Dr. Tom Brock.)
overlying layer of loosely arranged epithelium may resemble
the stellate reticulum of an ameloblastoma.
The most characteristic histopathologic feature of the
calcifying odontogenic cyst is the presence of variable
numbers of “ghost cells” within the epithelial component.
These eosinophilic ghost cells are altered epithelial cells that
are characterized by the loss of nuclei with preservation of
the basic cell outline (Fig. 15-45).
The nature of the ghost cell change is controversial. Some
believe that this change represents coagulative necrosis or
accumulation of enamel protein; others contend it is a form
of normal or aberrant keratinization of odontogenic epithe-
lium. Masses of ghost cells may fuse to form large sheets of
amorphous, acellular material. Calcification within the
ghost cells is common. This first appears as fine basophilic
granules that may increase in size and number to form
extensive masses of calcified material. Areas of an eosino-
philic matrix material that are considered by some authors
to represent dysplastic dentin (dentinoid) also may be
present adjacent to the epithelial component. This is
believed to be the result of an inductive effect by the
• Fig. 15-44  Peripheral Calcifying Odontogenic Cyst. Nodular
mass of the mandibular facial gingiva.

• Fig. 15-45  Calcifying Odontogenic Cyst. The cyst lining shows
ameloblastoma-like epithelial cells, with a columnar basal layer. Large
eosinophilic ghost cells are present within the epithelial lining.
• Fig. 15-46  Calcifying Odontogenic Cyst. Eosinophilic dentinoid
material is present adjacent to a sheet of ghost cells.
odontogenic epithelium on the adjacent mesenchymal
tissue (Fig. 15-46).
Several variants of the cystic type of calcifying odonto-
genic cyst are seen. In some cases, the epithelial lining
proliferates into the lumen so that the lumen is largely filled
with masses of ghost cells and dystrophic calcifications.
Multiple daughter cysts may be present within the fibrous
wall, and a foreign body reaction to herniated ghost cells
may be conspicuous.
In another variant, unifocal or multifocal epithelial pro-
liferation of the cyst lining into the lumen may resemble
ameloblastoma. These proliferations are intermixed with
varying numbers of ghost cells. These epithelial prolifera-
tions superficially resemble, but do not meet, the strict
histopathologic criteria for ameloblastoma.
About 20% of calcifying odontogenic cysts are associated
with odontomas. This variant is usually a unicystic lesion
that shows the features of a calcifying odontogenic cyst
together with those of a small complex or compound
odontoma.
Solid dentinogenic ghost cell tumors may occur intraos-
seously or extraosseously. The extraosseous forms appear to
CHAPTER 15  Odontogenic Cysts and Tumors 649
be more common. These show varying-sized islands of
odontogenic epithelium in a fibrous stroma. The epithelial
islands show peripheral palisaded columnar cells and central
stellate reticulum, which resemble ameloblastoma. Nests of
ghost cells, however, are present within the epithelium,
and juxtaepithelial dentinoid is commonly present. These
features differentiate this lesion from the peripheral
ameloblastoma.
The rare intraosseous variant is a solid tumor that con-
sists of ameloblastoma-like strands and islands of odonto-
genic epithelium in a mature fibrous connective tissue
stroma. Variable numbers of ghost cells and juxtaepithelial
dentinoid are present.
A small number of aggressive or malignant epithelial
odontogenic ghost cell tumors (ghost cell odontogenic
carcinoma) have been reported. These lesions have cellular
pleomorphism and mitotic activity with invasion of the
surrounding tissues.
Treatment and Prognosis
The prognosis for a patient with a calcifying odontogenic
cyst is good; only a few recurrences after simple enucleation
have been reported. Peripheral examples appear to have
the same prognosis as a peripheral ameloblastoma, with
a minimal chance of recurrence after simple surgical
excision.
When a calcifying odontogenic cyst is associated with
some other recognized odontogenic tumor, such as an ame-
loblastoma, the treatment and prognosis are likely to be the
same as for the associated tumor. Although few cases have
been reported, ghost cell odontogenic carcinomas appear to
have an unpredictable behavior. Recurrences are common,
and a few patients have died from either uncontrolled local
disease or metastases. An overall 5-year survival rate of 73%
has been calculated for reported cases.
◆ GLANDULAR ODONTOGENIC CYST
(SIALO-ODONTOGENIC CYST)
The glandular odontogenic cyst is a rare type of develop-
mental odontogenic cyst that can show aggressive behavior.
Although it is generally accepted as being of odontogenic
origin, it also shows glandular or salivary features that pre-
sumably are an indication of the pluripotentiality of odon-
togenic epithelium.
Clinical and Radiographic Features
The glandular odontogenic cyst occurs most commonly in
middle-aged adults, with a mean age of 46 to 51 years at
the time of diagnosis; rarely does it occur before the age of
20. Approximately 75% of reported cases have occurred in
the mandible. The cyst has a strong predilection for the
anterior region of the jaws, and many mandibular lesions
will cross the midline.
650 C H A P T E R 1 5 Odontogenic Cysts and Tumors

The size of the cyst can vary from small lesions less than propensity for recurrence, which is observed in approxi-
1 cm in diameter to large destructive lesions that may mately 30% of all cases. Recurrence appears to be more
involve most of the jaw. Small cysts may be asymptomatic; common among the lesions that present in a multilocular
however, large cysts often produce clinical expansion, which fashion. Because of its potentially aggressive nature and
sometimes can be associated with pain or paresthesia (Fig. tendency for recurrence, some authors have advocated en
15-47). bloc resection, particularly for multilocular lesions. Marsu-
Radiographically, the lesion presents as either a unilocu- pialization and decompression may be attempted for larger
lar or multilocular radiolucency. The margins of the radio- lesions to promote shrinkage prior to surgery.
lucency are usually well defined with a corticated rim.
◆ BUCCAL BIFURCATION CYST
Histopathologic Features
The buccal bifurcation cyst is an uncommon inflamma-
The glandular odontogenic cyst is lined by squamous epi- tory odontogenic cyst that characteristically develops on the
thelium of varying thickness. The interface between the buccal aspect of the mandibular first permanent molar,
epithelium and the fibrous connective tissue wall is gener- although some cases have involved the second molar. The
ally flat. The fibrous cyst wall is usually devoid of any pathogenesis of this cyst is uncertain. Some of these lesions
inflammatory cell infiltrate. The superficial epithelial cells have been associated with teeth that demonstrate buccal
that line the cyst cavity tend to be cuboidal to columnar, enamel extensions into the bifurcation area (see page 86).
resulting in an uneven hobnail and sometimes papillary Such extensions may predispose these teeth to buccal pocket
surface (Fig. 15-48). The surface layer often includes mucin- formation, which could then enlarge to form a cyst in
producing goblet cells, occasionally with the presence of
cilia. Glandular, ductlike spaces within the epithelial lining
are another characteristic finding. These spaces are lined by
cuboidal cells and often contain mucicarmine-positive fluid.
In focal areas, the epithelial lining cells may form spherical
nodules, similar to those seen in lateral periodontal cysts.
There is some histopathologic overlap between the fea-
tures of the glandular odontogenic cyst and those of some
intraosseous, low-grade, predominantly cystic mucoepider-
moid carcinomas (see page 457). In selected microscopic
fields, the microscopic features may be identical. Examina-
tion of multiple sections, however, usually permits the dif-
ferentiation of these lesions. Also, glandular odontogenic
cysts will not show MAML2 gene rearrangements, which
often are found in central mucoepidermoid carcinomas.

Treatment and Prognosis

• Fig. 15-48  Glandular Odontogenic Cyst. The cyst is lined by
Most cases of glandular odontogenic cyst have been treated stratified squamous epithelium that exhibits surface columnar cells with
by enucleation or curettage. However, this cyst shows a cilia. Numerous microcysts containing mucinous material are present.

A B

• Fig. 15-47  Glandular Odontogenic Cyst. A, Expansile lesion of the anterior mandible. B, The
panoramic radiograph shows a large multilocular radiolucency. (Courtesy of Dr. Cheng-Chung Lin.)

response to pericoronitis. It has been speculated that when
the tooth erupts, an inflammatory response may occur in
the surrounding follicular tissues that stimulates cyst
formation.
The term paradental cyst sometimes has been used syn-
onymously for the buccal bifurcation cyst. Such lesions
typically occur distal or buccal of partially erupted man-
dibular third molars with a history of pericoronitis. The
pathogenesis of the so-called paradental cyst also is uncer-
tain. However, the distinction of paradental cysts from sec-
ondarily inflamed dentigerous cysts is difficult, if not
impossible, in many instances (see page 632).
Clinical and Radiographic Features
The buccal bifurcation cyst typically occurs in children from
5 to 13 years of age. The patient has slight-to-moderate
tenderness on the buccal aspect of the mandibular first or
second molar, which may be in the process of erupting. The
patient often notes associated clinical swelling and a foul-
tasting discharge. Periodontal probing usually reveals pocket
formation on the buccal aspect of the involved tooth.
Around one-third of patients have been reported to have
bilateral involvement of the first molars.
Radiographs typically show a well-circumscribed uni-
locular radiolucency involving the buccal bifurcation and
root area of the involved tooth (Fig. 15-49). The average
size of the lucent defect is 1.2 cm, but the lesion may be as
large as 2.5 cm in diameter. An occlusal radiograph is most
helpful in demonstrating the buccal location of the lesion.
The root apices of the molar are characteristically tipped
toward the lingual mandibular cortex (Fig. 15-50). Many
• Fig. 15-49  Buccal Bifurcation Cyst. Well-circumscribed unilocu-
lar radiolucency superimposed on the roots of the mandibular first
permanent molar. (Courtesy of Dr. Michael Pharoah.)
CHAPTER 15  Odontogenic Cysts and Tumors 651
cases are associated with proliferative periostitis (see page
134) of the overlying buccal cortex, which is characterized
by a single or multiple layers of reactive bone formation.
Histopathologic Features
The microscopic features are nonspecific and show a cyst
that is lined by nonkeratinizing stratified squamous epithe-
lium with areas of hyperplasia. A prominent chronic inflam-
matory cell infiltrate is present in the surrounding connective
tissue wall.
Treatment and Prognosis
The buccal bifurcation cyst is usually treated by enucleation;
extraction of the associated tooth is unnecessary. Within 1
year of surgery, there is usually complete healing with nor-
malization of periodontal probing depths and radiographic
evidence of bone fill. Several reports have described cases
that resolved without surgery—either with no treatment at
all or by daily irrigation of the buccal pocket with saline
and hydrogen peroxide.
◆CARCINOMA ARISING IN
ODONTOGENIC CYSTS
Carcinoma arising within bone is a rare lesion that is essen-
tially limited to the jaws. Because the putative source of the
epithelium giving rise to the carcinoma is odontogenic,
these intraosseous jaw carcinomas are collectively known as
odontogenic carcinomas. Odontogenic carcinomas may
arise in an ameloblastoma, rarely from other odontogenic
tumors, de novo (without evidence of a preexisting lesion),
or from the epithelial lining of odontogenic cysts. Some
intraosseous mucoepidermoid carcinomas (see page 457)
also may arise from mucous cells lining a dentigerous cyst.
• Fig. 15-50  Buccal Bifurcation Cyst. Axial computed tomography
(CT) image showing a circumscribed radiolucency buccal to the roots
of the mandibular first molar. (Courtesy of Dr. Robert Clark.)
652 C H A P T E R 1 5 Odontogenic Cysts and Tumors
Most intraosseous carcinomas apparently arise in odon-
togenic cysts. Although infrequently documented in the
literature, carcinomatous transformation of the lining of an
odontogenic cyst may be more common than is generally
appreciated. Several studies have shown that 1% to 2% of
all oral cavity carcinomas seen in some oral and maxillofa-
cial pathology services may originate from odontogenic
cysts. The pathogenesis of carcinomas arising in odonto-
genic cysts is unknown. Occasionally, areas within the
lining of odontogenic cysts histopathologically demonstrate
varying degrees of epithelial dysplasia, and such changes
likely give rise to the carcinoma.
Clinical and Radiographic Features
Although carcinomas arising in cysts may be seen in patients
across a wide age range, they are encountered most often in
older patients. The mean reported age is 60 years. This
lesion is over twice as common in men as in women. Pain
and swelling are the most common complaints. However,
many patients have no symptoms, and the diagnosis of
carcinoma is made only after microscopic examination of a
presumed odontogenic cyst.
Radiographic findings may mimic those of any odonto-
genic cyst, although the margins of the radiolucent defect
are usually irregular and ragged. CT scans of the lesion may
demonstrate a destructive pattern that is not appreciated on
viewing plain radiographs. A lesion considered to be a resid-
ual periapical cyst is apparently the most common type
associated with carcinomatous transformation, although
routine periapical cysts can also exhibit malignant change.
These account for 60% of reported cases. In about 16% of
cases, the carcinoma appeared to have arisen in a dentiger-
ous cyst (Fig. 15-51). In one patient, the carcinoma was
thought to originate from a lateral periodontal cyst.
A number of examples of carcinoma arising in an OKC
also have been documented (Fig. 15-52). However, some
reported examples do not appear to have arisen in true
• Fig. 15-51  Carcinoma Arising in a Dentigerous Cyst. Radiolu-
cent lesion surrounding the crown of an impacted third molar in a
56-year-old woman. This was clinically considered to be a dentigerous
cyst. (Courtesy of Dr. Richard Ziegler.)
parakeratinized OKCs, but rather in orthokeratinized
odontogenic cysts.
Histopathologic Features
Most carcinomas arising in cysts histopathologically have
been well-differentiated or moderately well-differentiated
squamous cell carcinomas. Sometimes it is possible to
identify a transition from a normal-appearing cyst lining
to invasive squamous cell carcinoma (Figs. 15-53 and
15-54).
Treatment and Prognosis
The treatment of patients with carcinomas arising in cysts
has varied from local block excision to radical resection,
with or without radiation or adjunctive chemotherapy. The
prognosis is difficult to evaluate because most reports consist
of isolated cases. Metastases to regional lymph nodes have
been demonstrated in a few cases. One review showed an
overall 2-year survival rate of 62%, but the 5-year survival
rate dropped to 38%.
Before a given lesion can be accepted as an example of
primary intraosseous carcinoma, the possibility that the
tumor represents metastatic spread from an intraoral or
extraoral site must be ruled out by appropriate studies.
• Fig. 15-52  Carcinoma Arising in a Cyst. There is a massive
carcinoma of the mandible, with extension into the parotid gland, the
face, and the base of the brain. Nineteen years previously, a large
odontogenic keratocyst (OKC) with areas of epithelial dysplasia had
been removed from the ascending ramus. The patient had suffered
multiple recurrences, with eventual change into invasive carcinoma.

• Fig. 15-53  Carcinoma Arising in a Cyst. High-power view of a
dentigerous cyst from a 53-year-old man. The lining demonstrates
full-thickness epithelial dysplasia.
• Fig. 15-54  Carcinoma Arising in a Cyst. Same case as Fig.
15-53 showing islands of invasive epithelial cells in the cyst wall.
ODONTOGENIC TUMORS
Odontogenic tumors comprise a complex group of lesions
of diverse histopathologic types and clinical behavior. Some
of these lesions are true neoplasms and may rarely exhibit
malignant behavior. Others may represent tumorlike mal-
formations (hamartomas).
Odontogenic tumors, like normal odontogenesis, dem-
onstrate varying inductive interactions between odonto-
genic epithelium and odontogenic ectomesenchyme. This
ectomesenchyme was formerly referred to as mesenchyme
because it was thought to be derived from the mesodermal
layer of the embryo. It is now accepted that this tissue dif-
ferentiates from the ectodermal layer in the cephalic portion
of the embryo. Tumors of odontogenic epithelium are
composed only of odontogenic epithelium without any par-
ticipation of odontogenic ectomesenchyme.
Other odontogenic neoplasms, sometimes referred to as
mixed odontogenic tumors, are composed of odontogenic
epithelium and ectomesenchymal elements. Dental hard
tissue may or may not be formed in these lesions.
CHAPTER 15  Odontogenic Cysts and Tumors 653
• BOX 15-4 Classification of Odontogenic Tumors
I. Tumors of odontogenic epithelium
A. Ameloblastoma
1. Malignant ameloblastoma
2. Ameloblastic carcinoma
B. Clear cell odontogenic carcinoma
C. Adenomatoid odontogenic tumor
D. Calcifying epithelial odontogenic tumor
E. Squamous odontogenic tumor
II. Mixed odontogenic tumors
A. Ameloblastic fibroma
B. Ameloblastic fibro-odontoma
C. Ameloblastic fibrosarcoma
D. Odontoameloblastoma
E. Compound odontoma
F. Complex odontoma
III. Tumors of odontogenic ectomesenchyme
A. Odontogenic fibroma
B. Granular cell odontogenic tumor
C. Odontogenic myxoma
D. Cementoblastoma
A third group, tumors of odontogenic ectomesen-
chyme, is composed principally of ectomesenchymal ele-
ments. Although odontogenic epithelium may be included
within these lesions, it does not appear to play any essential
role in their pathogenesis.
Box 15-4 presents categories of odontogenic tumors
modified from the 2005 WHO classification.
Because many of these lesions are quite rare, it is some-
times difficult to assess certain epidemiologic features accu-
rately, as well as recommendations regarding treatment. It
should be kept in mind that reports in the literature may
be biased due to geopolitical/economic variations in sub-
mission of biopsies or the tendency of journal editors to
publish reports of lesions that are unusual or aggressive.
TUMORS OF ODONTOGENIC
EPITHELIUM
Epithelial odontogenic tumors are composed of odonto-
genic epithelium without participation of odontogenic
ectomesenchyme. Several distinctly different tumors are
included in the group; ameloblastoma is the most impor-
tant and common of them.
◆ AMELOBLASTOMA
The ameloblastoma is the most common clinically signifi-
cant odontogenic tumor. Its relative frequency equals the
combined frequency of all other odontogenic tumors,
excluding odontomas. Ameloblastomas are tumors of odon-
togenic epithelial origin. Theoretically, they may arise from
rests of dental lamina, from a developing enamel organ,
from the epithelial lining of an odontogenic cyst, or from
the basal cells of the oral mucosa. Ameloblastomas are
654 C H A P T E R 1 5 Odontogenic Cysts and Tumors

slow-growing, locally invasive tumors that run a benign expansion is frequently present. Resorption of the roots of
course in most cases. They typically have been described as teeth adjacent to the tumor is common. In many cases an
having three different clinicoradiographic presentations, unerupted tooth, most often a mandibular third molar, is
which deserve separate consideration because of potentially associated with the radiolucent defect. Solid ameloblasto-
differing therapeutic considerations and prognosis: mas may radiographically appear as unilocular radiolucent
1. Conventional solid or multicystic (about 75% to 86% defects, which may resemble almost any type of cystic lesion
of all cases) (Fig. 15-62). The margins of these radiolucent lesions,
2. Unicystic (about 13% to 21% of all cases) however, often show irregular scalloping. Although the
3. Peripheral (extraosseous) (about 1% to 4% of all cases) radiographic features, particularly of the typical multilocu-
lar defect, may be highly suggestive of ameloblastoma, a
CONVENTIONAL SOLID OR MULTICYSTIC
INTRAOSSEOUS AMELOBLASTOMA
Clinical and Radiographic Features
Conventional solid or multicystic intraosseous amelo-
blastoma is encountered in patients across a wide age range.
It is rare in children younger than age 10 and relatively
uncommon in the 10- to 19-year-old group. The tumor
shows an approximately equal prevalence in the third to
seventh decades of life. There is no significant sex predilec-
tion. Some studies indicate a greater frequency in blacks;
others show no racial predilection. About 80% to 85% of
conventional ameloblastomas occur in the mandible, most
often in the molar-ascending ramus area. About 15% to
20% of ameloblastomas occur in the maxilla, usually in the
posterior regions (Fig. 15-55). The tumor is often asymp-
tomatic, and smaller lesions are detected only during a
radiographic examination. A painless swelling or expansion
of the jaw is the usual clinical presentation (Figs. 15-56 and
15-57). If untreated, then the lesion may grow slowly to
massive or grotesque proportions (Fig. 15-58). Pain and
paresthesia are uncommon, even with large tumors.
The most typical radiographic feature is that of a multi-
locular radiolucent lesion, although one large international
study suggested that a unilocular presentation was just as
likely. Multilocular lesions are described as having a “soap
bubble” appearance (when the radiolucent loculations are • Fig. 15-56  Ameloblastoma. Large expansile mass of the anterior
mandible. (Courtesy of Dr. Michael Tabor.)
large) or as being “honeycombed” (when the loculations are
small) (Figs. 15-59 to 15-61). Buccal and lingual cortical

6% 1% 6%

66% 11% 10%

• Fig. 15-57  Ameloblastoma.Prominent expansion of the lingual

• Fig. 15-55  Ameloblastoma. Relative distribution of ameloblasto- alveolus caused by a large ameloblastoma of the mandibular
mas in the jaws. symphysis.

variety of odontogenic and nonodontogenic lesions may
show similar radiographic features (see Appendix).
One form of ameloblastoma that does not have these
characteristic features is the desmoplastic ameloblastoma, a
variant that Eversole and colleagues documented initially in
the literature in 1984. The desmoplastic ameloblastoma has
a marked predilection to occur in the anterior regions of
• Fig. 15-58  Ameloblastoma. Massive tumor of the anterior man-
dible. (Courtesy of Dr. Ronald Baughman.)
• Fig. 15-60  Ameloblastoma. Periapical films showing the “honeycombed” appearance. (Courtesy of
Dr. John Hann.)
CHAPTER 15  Odontogenic Cysts and Tumors 655
the jaws, with equal distribution between the mandible and
the maxilla. Radiographically, this type may not suggest the
diagnosis of ameloblastoma; the majority of these tumors
resemble a fibro-osseous lesion because of their mixed radio-
lucent and radiopaque appearance (Fig. 15-63). This mixed
radiographic appearance is due to osseous metaplasia within
the dense fibrous septa that characterize the lesion, not
because the tumor itself is producing a mineralized product.
Histopathologic Features
Conventional solid or multicystic intraosseous ameloblasto-
mas show a remarkable tendency to undergo cystic change;
grossly, most tumors have varying combinations of cystic
and solid features. The cysts may be seen only at the micro-
scopic level or may be present as multiple large cysts that
include most of the tumor. Several microscopic subtypes of
• Fig. 15-59  Ameloblastoma. Large multilocular lesion involving the
mandibular angle and ascending ramus. The large loculations show
the “soap bubble” appearance. An unerupted third molar has been
displaced high into the ramus.
656 C H A P T E R 1 5 Odontogenic Cysts and Tumors
• Fig. 15-61  Ameloblastoma. Destructive radiolucent lesion with
root resorption of the associated posterior teeth. (Courtesy of Dr. Louis
Beto.)
• Fig. 15-62  Ameloblastoma. This small unilocular radiolucency
lesion could easily be mistaken for a lateral periodontal cyst. (Courtesy
of Dr. Tony Traynham.)
conventional ameloblastoma are recognized, but these
microscopic patterns generally have little bearing on the
behavior of the tumor. Large tumors often show a combina-
tion of microscopic patterns.
The follicular and plexiform patterns are the most
common. Less common histopathologic patterns include
the acanthomatous, granular cell, desmoplastic, and
basal cell types.
Follicular Pattern
The follicular histopathologic pattern is the most common
and recognizable. Islands of epithelium resemble enamel
organ epithelium in a mature fibrous connective tissue
stroma. The epithelial nests consist of a core of loosely
• Fig. 15-63  Desmoplastic Ameloblastoma. Large mixed radiolu-
cent and radiopaque lesion of the anterior and right body of the
mandible. (Courtesy of Dr. Román Carlos.)
• Fig. 15-64  Ameloblastoma (Follicular Pattern). Multiple islands
of odontogenic epithelium demonstrating peripheral columnar differen-
tiation with reverse polarization. The central zones resemble stellate
reticulum and exhibit foci of cystic degeneration.
arranged angular cells resembling the stellate reticulum of
an enamel organ. A single layer of tall columnar ameloblast-
like cells surrounds this central core. The nuclei of these
cells are located at the opposite pole to the basement mem-
brane (reversed polarity). In other areas, the peripheral
cells may be more cuboidal and resemble basal cells. Cyst
formation is common and may vary from microcysts, which
form within the epithelial islands, to large macroscopic
cysts, which may be several centimeters in diameter (Figs.
15-64 and 15-65). If an incisional biopsy is taken from such
an area, an inappropriate diagnosis of “unicystic ameloblas-
toma” may be rendered by the pathologist.
Plexiform Pattern
The plexiform type of ameloblastoma consists of long, anas-
tomosing cords or larger sheets of odontogenic epithelium.
The cords or sheets of epithelium are bounded by columnar
or cuboidal ameloblast-like cells surrounding more loosely

• Fig. 15-65  Ameloblastoma (Follicular Pattern). This high-power
photomicrograph highlights the peripheral columnar cells exhibiting
reverse polarization.
• Fig. 15-66  Ameloblastoma (Plexiform Pattern). Anastomosing
cords of odontogenic epithelium.
arranged epithelial cells. The supporting stroma tends to be
loosely arranged and vascular. Cyst formation is relatively
uncommon in this variety. When it occurs, it is more often
associated with stromal degeneration rather than cystic
change within the epithelium (Fig. 15-66).
Acanthomatous Pattern
When extensive squamous metaplasia, often associated with
keratin formation, occurs in the central portions of the
epithelial islands of a follicular ameloblastoma, the term
acanthomatous ameloblastoma is sometimes applied. This
change does not indicate a more aggressive course for the
lesion; histopathologically, however, such a lesion may be
confused with squamous cell carcinoma or squamous odon-
togenic tumor (Fig. 15-67).
Granular Cell Pattern
Ameloblastomas may sometimes show transformation of
groups of lesional epithelial cells to granular cells. These
cells have abundant cytoplasm filled with eosinophilic gran-
ules that resemble lysosomes ultrastructurally and histo-
chemically. Although originally considered to represent an
CHAPTER 15  Odontogenic Cysts and Tumors 657
• Fig. 15-67  Ameloblastoma (Acanthomatous Pattern). Islands
of ameloblastoma demonstrating central squamous differentiation.
• Fig. 15-68  Ameloblastoma (Granular Cell Variant). Tumor
island exhibiting central cells with prominent granular cytoplasm.
aging or degenerative change in long-standing lesions, this
variant has been seen in young patients. When this granular
cell change is extensive in an ameloblastoma, the designa-
tion of granular cell ameloblastoma is appropriate (Fig.
15-68).
Desmoplastic Pattern
This type of ameloblastoma contains small islands and cords
of odontogenic epithelium in a densely collagenized stroma.
Immunohistochemical studies have shown increased pro-
duction of the cytokine known as transforming growth
factor-β (TGF-β) in association with this lesion, suggesting
that this may be responsible for the desmoplasia. Peripheral
columnar ameloblast-like cells are inconspicuous about the
epithelial islands (Fig. 15-69).
Basal Cell Pattern
The basal cell variant of ameloblastoma is the least common
type. These lesions are composed of nests of uniform basa-
loid cells, and they histopathologically are very similar to
658 C H A P T E R 1 5 Odontogenic Cysts and Tumors
basal cell carcinoma of the skin. No stellate reticulum is
present in the central portions of the nests. The peripheral
cells about the nests tend to be cuboidal rather than colum-
nar (Fig. 15-70).
Treatment and Prognosis
Patients with conventional solid or multicystic intraosseous
ameloblastomas have been treated by a variety of means.
These range from simple enucleation and curettage to en
bloc resection (Fig. 15-71). The optimal method of treat-
ment has been the subject of controversy for many years.
The conventional ameloblastoma tends to infiltrate between
intact cancellous bone trabeculae at the periphery of the
lesion before bone resorption becomes radiographically
evident. Therefore, the actual margin of the tumor often
extends beyond its apparent radiographic or clinical margin.
Attempts to remove the tumor by curettage often leave
small islands of tumor within the bone, which later manifest
as recurrences. Recurrence rates of 50% to 90% have been
reported in various studies after curettage. Recurrence often
• Fig. 15-69  Ameloblastoma (Desmoplastic Variant). Thin cords
of ameloblastic epithelium within a dense fibrous connective tissue
stroma.
A B
• Fig. 15-71  Ameloblastoma. A, Gross photograph of a mandibular resection specimen. B, The
radiograph of the specimen shows a large radiolucent defect associated with an inferiorly displaced third
molar. (Courtesy of Dr. Mary Richardson.)
takes many years to become clinically manifest, and 5-year
disease-free periods do not indicate a cure.
Marginal resection is the most widely used treatment,
but recurrence rates of up to 15% have been reported after
marginal or block resection. Some surgeons advocate a more
conservative approach to treatment by planning surgery
after careful evaluation of CT scans of the tumor. Removal
of the tumor, followed by peripheral ostectomy, often
reduces the need for extensive reconstructive surgery. Some
tumors may not be amenable to this approach because of
their size or growth pattern.
Other surgeons advocate that the margin of the resection
should be at least 1.0 to 2.0 cm past the radiographic limits
of the tumor. Ameloblastomas of the posterior maxilla are
particularly dangerous because of the difficulty of obtaining
an adequate surgical margin around the tumor. Orbital
invasion by maxillary ameloblastomas occasionally has been
described. Although some studies suggest that the amelo-
blastoma may be radiosensitive, radiation therapy has
seldom been used as a treatment modality because of the
intraosseous location of the tumor and the potential for
• Fig. 15-70  Ameloblastoma (Basal Cell Variant). Islands of
hyperchromatic basaloid cells with peripheral palisading.
 CHAPTER 15  Odontogenic Cysts and Tumors 659

secondary radiation-induced malignancy developing in a

relatively young patient population.
The conventional ameloblastoma is a persistent, infiltra-
tive neoplasm that very seldom may kill the patient by
progressive spread to involve vital structures. Most of these
tumors, however, are not life-threatening lesions. Rarely, an
ameloblastoma exhibits frank malignant behavior. These are
discussed separately.

UNICYSTIC AMELOBLASTOMA
The unicystic ameloblastoma has for several decades been
given separate consideration based on its clinical, radio-
graphic, and pathologic features. Although its response to
treatment in reports from the 1970s and 1980s suggested
that this lesion might behave in a less aggressive fashion,
recent reports have disputed this concept. Unicystic amelo-
blastomas account for 10% to 46% of all intraosseous • Fig. 15-72  Unicystic Ameloblastoma. A large radiolucency asso-
ameloblastomas in various studies. Whether the unicystic ciated with the crown of the developing mandibular third molar. (Cour-
ameloblastoma originates de novo as a neoplasm or whether tesy of Dr. Antonia Kolokythas.)
it is the result of neoplastic transformation of nonneoplastic
cyst epithelium has been long debated. Both mechanisms
probably occur, but proof of which is involved in an indi-
vidual patient is virtually impossible to obtain.

Clinical and Radiographic Features

Unicystic ameloblastomas are seen most often in younger
patients, with about 50% of all such tumors diagnosed
during the second decade of life. The average age in one
large series was 23 years. More than 90% of unicystic ame-
loblastomas are found in the mandible, usually in the pos-
terior regions. The lesion is often asymptomatic, although
large lesions may cause a painless swelling of the jaws.
In many patients, this lesion typically appears as a cir-
cumscribed radiolucency that surrounds the crown of an
unerupted mandibular third molar (Figs. 15-72 and 15-73),
clinically resembling a dentigerous cyst. Other tumors
simply appear as sharply defined radiolucent areas and are
usually considered to be a primordial, radicular, or residual
cyst, depending on the relationship of the lesion to teeth in
the area. In some instances, the radiolucent area may have
scalloped margins but is still a unicystic ameloblastoma.
Whether a unicystic ameloblastoma can have a truly mul-
tilocular radiographic presentation is arguable.
The surgical findings may also suggest that the lesion in
question is a cyst, and the diagnosis of ameloblastoma is
made only after microscopic study of the specimen.
Histopathologic Features
Three histopathologic variants of unicystic ameloblastoma
have been described. In the first type (luminal unicystic
ameloblastoma), the tumor is confined to the luminal
surface of the cyst. The lesion consists of a fibrous cyst wall
with a lining composed totally or partially of ameloblastic
epithelium. The lining demonstrates a basal layer of
• Fig. 15-73  Unicystic Ameloblastoma (Intraluminal Plexiform
Type). Coronal computed tomography (CT) image that shows a large
cystic lesion with an intraluminal mass arising from the cyst wall
(arrow).
columnar or cuboidal cells with hyperchromatic nuclei that
show reverse polarity and basilar cytoplasmic vacuolization
(Fig. 15-74). The upper epithelial cells are loosely cohesive
and resemble stellate reticulum. This finding does not seem
to be related to inflammatory edema.
In the second microscopic variant, one or more nodules
of ameloblastoma project from the cystic lining into the
lumen of the cyst. This type is called an intraluminal uni-
cystic ameloblastoma. These nodules may be relatively
small or largely fill the cystic lumen. In some cases, the
nodule of tumor that projects into the lumen demonstrates
an edematous, plexiform pattern that resembles the plexi-
form pattern seen in conventional ameloblastomas (Fig.
15-75). These lesions are sometimes referred to as plexi-
form unicystic ameloblastomas. The intraluminal cellular
proliferation does not always meet the strict histopathologic
660 C H A P T E R 1 5 Odontogenic Cysts and Tumors
• Fig. 15-74  Unicystic Ameloblastoma (Luminal Type). The cyst
is lined by ameloblastic epithelium showing a hyperchromatic, polar-
ized basal layer. The overlying epithelial cells are loosely cohesive and
resemble stellate reticulum.
• Fig. 15-75  Unicystic Ameloblastoma (Intraluminal Plexiform
Type). Photomicrograph of an intraluminal mass arising from the cyst
wall. The inset shows the intraluminal mass at higher magnification.
criteria for ameloblastoma, and this may be secondary to
inflammation that nearly always accompanies this pattern.
Typical ameloblastoma, however, may be found in other,
less inflamed parts of the specimen.
In the third variant, known as mural unicystic amelo-
blastoma, the fibrous wall of the cyst is infiltrated by typical
follicular or plexiform ameloblastoma. The extent and
depth of the ameloblastic infiltration may vary considerably.
With any presumed unicystic ameloblastoma, multiple sec-
tions through many levels of the specimen are necessary to
rule out the possibility of mural invasion of tumor cells
(Fig. 15-76).
Treatment and Prognosis
The clinical and radiographic findings in most cases of
unicystic ameloblastoma suggest that the lesion is an odon-
togenic cyst. These tumors are usually treated as cysts by
enucleation. The diagnosis of ameloblastoma is made only
after microscopic examination of the presumed cyst. If the
ameloblastic elements are confined to the lumen of the cyst
• Fig. 15-76  Unicystic Ameloblastoma (Mural Type). The epithe-
lial lining of the cystic component can be seen on the left edge of the
photomicrograph. Islands of follicular ameloblastoma are infiltrating
into the fibrous connective tissue wall on the right.
with or without intraluminal tumor extension, then the cyst
enucleation has probably been adequate treatment. The
patient, however, should be kept under long-term follow-up.
If the specimen shows extension of the tumor into the
fibrous cyst wall for any appreciable distance, then subse-
quent management of the patient is more controversial.
Some surgeons believe that local resection of the area is
indicated as a prophylactic measure; others prefer to keep
the patient under close radiographic observation and delay
further treatment until there is evidence of recurrence.
Recurrence rates of 10% to 20% were described after
enucleation and curettage of unicystic ameloblastomas in
many of the earlier series of cases. This range is considerably
less than the 50% to 90% recurrence rates noted after curet-
tage of conventional solid and multicystic intraosseous
ameloblastomas. A systematic review of the literature before
2005 determined that 30% of these lesions recurred after
enucleation, and a recent single-center study showed an
identical recurrence rate of 60% after enucleation for both
solid and unicystic ameloblastoma. These findings suggest
that this lesion may not be as innocuous as previously
thought. Alternatively, it is possible that some of those
tumors that are designated as “unicystic” may, in fact, have
a more characteristic invasive component that has not been
detected histopathologically because it is essentially impos-
sible to examine these lesions in every 360-degree plane of
section.
PERIPHERAL (EXTRAOSSEOUS)
AMELOBLASTOMA
The peripheral ameloblastoma is uncommon and accounts
for about 1% to 4% of all ameloblastomas. This tumor
probably arises from rests of dental lamina beneath the oral
mucosa or from the basal epithelial cells of the surface epi-
thelium. Histopathologically, these lesions have the same
features as the intraosseous form of the tumor.

• Fig. 15-77  Peripheral Ameloblastoma. Sessile gingival mass.
(Courtesy of Dr. Dean K. White.)
Clinical Features
The peripheral ameloblastoma is usually a painless, nonul-
cerated sessile or pedunculated gingival or alveolar mucosal
lesion. The clinical features are non-specific, and most
lesions are clinically considered to represent a fibroma or
pyogenic granuloma. Most examples are smaller than
1.5 cm, but larger lesions have been reported (Fig. 15-77).
The tumor has been found in patients across a wide age
range, but most are seen in middle-aged persons, with an
average reported age of 52 years.
Peripheral ameloblastomas are most commonly found on
the posterior gingival and alveolar mucosa, and they are
somewhat more common in mandibular than in maxillary
areas. In some cases, the superficial alveolar bone becomes
slightly eroded, but significant bone involvement does not
occur. A few examples of a microscopically identical lesion
have been reported in the buccal mucosa at some distance
from the alveolar or gingival soft tissues.
Histopathologic Features
Peripheral ameloblastomas have islands of ameloblastic epi-
thelium that occupy the lamina propria underneath the
surface epithelium (Fig. 15-78). The proliferating epithe-
lium may show any of the features described for the intraos-
seous ameloblastoma; plexiform or follicular patterns are
the most common. Connection of the tumor with the basal
layer of the surface epithelium is seen in about 50% of cases.
This may represent origin of the tumor from the basal layer
of the epithelium in some cases, but in other instances the
tumor could develop in the gingival connective tissue and
merge with the surface epithelium.
Basal cell carcinomas of the gingival mucosa have been
reported, but most of these would be designated best as
peripheral ameloblastomas. A peripheral odontogenic
fibroma may be confused microscopically with a peripheral
ameloblastoma, particularly if a prominent epithelial com-
ponent is present in the former. The presence of dysplastic
dentin or cementum-like elements in the peripheral
CHAPTER 15  Odontogenic Cysts and Tumors 661
• Fig. 15-78  Peripheral Ameloblastoma. Interconnecting cords of
ameloblastic epithelium filling the lamina propria.
odontogenic fibroma and the lack of peripheral columnar
epithelial cells showing reverse polarity of their nuclei
should serve to distinguish the two lesions.
Treatment and Prognosis
Unlike the intraosseous ameloblastoma, the peripheral ame-
loblastoma shows an innocuous clinical behavior. Patients
respond well to local surgical excision. Although local recur-
rence has been noted in 15% to 20% of cases, further local
excision almost always results in a cure. Several examples of
malignant change in a peripheral ameloblastoma have been
reported, but this is rare.
◆MALIGNANT AMELOBLASTOMA AND
AMELOBLASTIC CARCINOMA
Rarely, an ameloblastoma exhibits frank malignant behavior
with development of metastases. The frequency of malignant
behavior in ameloblastomas is difficult to determine but
probably occurs in far less than 1% of all ameloblastomas.
The terminology for these lesions is somewhat confusing,
but should not be considered controversial. The term malig-
nant ameloblastoma is used for a tumor that shows the
histopathologic features of ameloblastoma, both in the
primary tumor and in the metastatic deposits. This is a very
rare neoplasm, with fewer than 30 well-documented cases
described in the literature. The term ameloblastic carcinoma
should be reserved for an ameloblastoma that has cytologic
features of malignancy in the primary tumor, in a recur-
rence, or in any metastatic deposit. This is also a rare condi-
tion, although approximately 200 cases have been reported.
These lesions may follow a markedly aggressive local course,
but metastases do not necessarily occur.
Clinical and Radiographic Features
Malignant ameloblastomas have been observed in patients
who range in age from 6 to 61 years (mean age, 30 years),
662 C H A P T E R 1 5 Odontogenic Cysts and Tumors
A B
• Fig. 15-79  Ameloblastic Carcinoma. A, Rapidly growing tumor showing prominent labial expansion
of the mandible in the incisor and premolar area. B, The panoramic radiograph shows irregular destruction
of the mandible. (From Neville BW, Damm DD, White DK: Color atlas of clinical oral pathology, ed 2,
Hamilton, 1999, BC Decker.)
and no sex predilection is seen. For patients with docu-
mented metastases, the interval between the initial treat-
ment of the ameloblastoma and first evidence of metastasis
varies from 3 to 45 years. In nearly one-third of cases,
metastases do not become apparent until 10 years after
treatment of the primary tumor. Ameloblastic carcinomas,
in contrast, tend to develop later in life, with the mean
age at diagnosis typically being in the sixth decade of life.
Men are affected twice as frequently as women.
Metastases from ameloblastomas are found most often
in the lungs. These have sometimes been regarded as aspira-
tion or implant metastases. However, the peripheral loca-
tion of some of these lung metastases suggests that they
must have occurred by blood or lymphatic routes rather
than aspiration.
Cervical lymph nodes are the second most common site
for metastasis of an ameloblastoma. Spread to vertebrae,
other bones, and viscera has also occasionally been
confirmed.
The radiographic findings of malignant ameloblastomas
may be essentially the same as those in typical nonmetasta-
sizing ameloblastomas. Ameloblastic carcinomas are often
more aggressive lesions, with ill-defined margins and corti-
cal destruction (Fig. 15-79).
Histopathologic Features
With malignant ameloblastomas, the primary jaw tumor
and the metastatic deposits show no microscopic features
that differ from those of ameloblastomas with a com-
pletely benign local course. With ameloblastic carcinomas,
the metastatic deposit or primary tumor shows the
microscopic pattern of ameloblastoma in addition to
cytologic features of malignancy. These include an increased
nuclear-to-cytoplasmic ratio, nuclear hyperchromatism,
and the presence of mitoses (Fig. 15-80). Necrosis in
tumor islands and areas of dystrophic calcification may
also be present.
Treatment and Prognosis
The prognosis of patients with malignant ameloblastomas
appears to be poor, but the paucity of documented cases
with long-term follow-up does not permit accurate assump-
tions to be made. About 50% of the patients with docu-
mented metastases and long-term follow-up have died of
their disease. Lesions designated as ameloblastic carcinoma
have demonstrated a uniformly aggressive clinical course,
with perforation of the cortical plates of the jaw and exten-
sion of the tumor into adjacent soft tissues.
◆ CLEAR CELL ODONTOGENIC
CARCINOMA (CLEAR CELL
ODONTOGENIC TUMOR)
Clear cell odontogenic carcinoma is a rare jaw tumor that
was first described in 1985. To date, approximately 80
examples have been documented. The tumor appears to be
of odontogenic origin, but its histogenesis is uncertain.
Histochemical and ultrastructural studies have suggested
that the clear cells, which are the prominent feature of this
neoplasm, may have similarities to glycogen-rich presecre-
tory ameloblasts. Recent molecular studies, however, have
identified rearrangements of the EWSR1 gene in clear cell
odontogenic carcinoma. This genetic alteration can be
found in a variety of tumors, but it is often seen in hyalin-
izing clear cell carcinoma, a rare salivary gland malignancy.
Consequently some authors have postulated that perhaps
some clear cell odontogenic carcinomas may represent an
intraosseous variant of hyalinizing clear cell carcinoma.
Clinical and Radiographic Features
The clear cell odontogenic carcinoma exhibits a variable
clinical pattern. A wide age range (from 14 to 89 years of
age) has been described, but most cases are diagnosed in

• Fig. 15-80  Ameloblastic Carcinoma. Ameloblastic epithelium
demonstrating hyperchromatism, pleomorphism, and numerous
mitotic figures.
• Fig. 15-81  Clear Cell Odontogenic Carcinoma. A radiolucent
defect at the apex of the mandibular first molar. (Courtesy of Dr. John
Werther.)
patients older than age 50. Nearly 80% of the lesions
develop in the mandible. Some patients complain of pain
or lower lip paresthesia, and bony swelling may be present.
Other patients are relatively symptom free. Approximately
60% of patients will have evidence of soft tissue involve-
ment by the tumor at the time of diagnosis because the
lesion perforates bone.
Radiographically, the lesions appear as unilocular or
multilocular radiolucencies. The margins of the radiolu-
cency are often somewhat ill-defined or irregular (Fig.
15-81).
Histopathologic Features
Three histopathologic patterns have been described for clear
cell odontogenic carcinoma. The biphasic pattern consists
of varying-sized nests of epithelial cells, with a clear or
faintly eosinophilic cytoplasm admixed with more eosino-
philic polygonal epithelial cells (Fig. 15-82). The second
pattern is more monophasic, characterized by only clear
cells that are arranged in nests and cords. Thin strands of
CHAPTER 15  Odontogenic Cysts and Tumors 663
• Fig. 15-82  Clear Cell Odontogenic Carcinoma. Hyperchromatic
epithelial nests including clusters of cells with abundant clear
cytoplasm.
• Fig. 15-83  Clear Cell Odontogenic Carcinoma. Tumor island
demonstrating cells with a clear cytoplasm. Note the peripheral colum-
nar differentiation.
hyalinized connective tissue often separate the clear cell
nests. The third pattern has a resemblance to ameloblastoma
in that the peripheral cells of the clear cell islands may
infrequently demonstrate palisading (Fig. 15-83). Often the
lesional cells do not exhibit a significant degree of nuclear
or cytologic pleomorphism. Furthermore, mitoses are gen-
erally sparse and necrosis is not a prominent feature. The
clear cells contain small amounts of glycogen, but mucin
stains are negative. In some cases, islands more typical of
ameloblastoma are interspersed among the other tumor
elements.
Hyalinizing clear cell carcinoma resembles clear cell
odontogenic carcinoma, and although the salivary gland
tumor develops in the soft tissues, for those lesions that have
significant bone destruction, it may be difficult to identify
the site of origin. Clear cell odontogenic carcinoma also
may be difficult to distinguish from intraosseous mucoepi-
dermoid carcinoma with a prominent clear cell component,
although the negative mucin stains are consistent with the
former. Amyloid stains would confirm the diagnosis of clear
cell variant of the calcifying epithelial odontogenic tumor,
because amyloid would not be present in clear cell
664 C H A P T E R 1 5 Odontogenic Cysts and Tumors

odontogenic tumor. A metastatic clear cell neoplasm, such

as a renal cell carcinoma, clear cell breast carcinoma, or clear
cell melanoma, may also need to be ruled out before the
diagnosis of clear cell odontogenic carcinoma can be 2% 9% 53%
established.

Treatment and Prognosis

Clear cell odontogenic carcinomas largely demonstrate an
aggressive clinical course, with invasion of contiguous struc- 2% 7% 27%
tures and a significant tendency to recur, particularly if the
initial treatment is enucleation or curettage. Most patients
require fairly radical surgery. Metastatic involvement of
regional lymph nodes has been documented in 20% to 25%
of these patients, and pulmonary metastases have been • Fig. 15-84  Adenomatoid Odontogenic Tumor. Relative distribu-
tion of adenomatoid odontogenic tumor in the jaws.
described as well.

◆ ADENOMATOID ODONTOGENIC TUMOR

The adenomatoid odontogenic tumor represents 2% to
7% of all odontogenic tumors, and more than 750 examples
have been reported in the literature. Although this lesion
was formerly considered to be a variant of the ameloblas-
toma and was designated as “adenoameloblastoma,” its
clinical features and biologic behavior indicate that it is a
separate entity. Postulated histogenetic sources of the tumor
cells have included enamel organ epithelium, reduced
enamel epithelium, and rests of Malassez; however, some
investigators recently have suggested that the lesion arises
from remnants of dental lamina associated with the guber-
nacular cord.
• Fig. 15-85  Adenomatoid Odontogenic Tumor. Radiolucent
lesion involving an unerupted mandibular first premolar. In contrast to
Clinical and Radiographic Features the usual dentigerous cyst, the radiolucency extends almost to the
apex of the tooth. (Courtesy of Dr. Tony Traynham.)
Adenomatoid odontogenic tumors are largely limited to
younger patients, and two-thirds of all cases are diagnosed
when patients are 10 to 19 years of age. This tumor is defi- type of adenomatoid odontogenic tumor may be impossible
nitely uncommon in a patient older than age 30. It has a to differentiate radiographically from the more common
striking tendency to occur in the anterior portions of the dentigerous cyst. The radiolucency associated with the fol-
jaws and is found twice as often in the maxilla as in the licular type of adenomatoid odontogenic tumor sometimes
mandible (Fig. 15-84). Females are affected about twice as extends apically along the root past the cementoenamel
often as males. junction. This feature may help to distinguish an adeno­
Most adenomatoid odontogenic tumors are relatively matoid odontogenic tumor from a dentigerous cyst
small. They seldom exceed 3 cm in greatest diameter, (Fig. 15-85).
although a few large lesions have been reported. Peripheral Less often the adenomatoid odontogenic tumor is a well-
(extraosseous) forms of the tumor are also encountered but delineated unilocular radiolucency that is not related to an
are rare. These usually appear as small, sessile masses on the unerupted tooth, but rather is located between the roots of
facial gingiva of the maxilla. Clinically, these lesions cannot erupted teeth (extrafollicular type) (Fig. 15-86).
be differentiated from the common gingival fibrous lesions. The lesion may appear completely radiolucent; often,
Adenomatoid odontogenic tumors are frequently asymp- however, it contains fine (snowflake) calcifications (Fig.
tomatic and are discovered during the course of a routine 15-87). This feature may be helpful in differentiating the
radiographic examination or when films are made to deter- adenomatoid odontogenic tumor from a dentigerous cyst.
mine why a tooth has not erupted. Larger lesions cause a
painless expansion of the bone. Histopathologic Features
In about 75% of cases, the tumor appears as a circum-
scribed, unilocular radiolucency that involves the crown of The adenomatoid odontogenic tumor is a well-defined
an unerupted tooth, most often a canine. This follicular lesion that is usually surrounded by a thick, fibrous capsule.

• Fig. 15-86  Adenomatoid Odontogenic Tumor. A small radiolu-
cency is present between the roots of the lateral incisor and canine.
(Courtesy of Dr. Ramesh Narang.)
• Fig. 15-87  Adenomatoid Odontogenic Tumor. Well-defined
pericoronal radiolucency enveloping the maxillary right lateral incisor in
a 14-year-old male. Note the subtle snowflake-like calcifications within
the lesion. (Courtesy of Dr. Jason Barker.)
CHAPTER 15  Odontogenic Cysts and Tumors 665
When the lesion is bisected, the central portion of the
tumor may be essentially solid or may show varying degrees
of cystic change (Fig. 15-88).
Microscopically, the tumor is composed of spindle-
shaped epithelial cells that form sheets, strands, or whorled
masses of cells in a scant fibrous stroma. The epithelial cells
may form rosettelike structures about a central space, which
may be empty or contain small amounts of eosinophilic
material. This material may stain for amyloid.
The tubular or ductlike structures, which are the charac-
teristic feature of the adenomatoid odontogenic tumor, may
be prominent, scanty, or even absent in a given lesion. These
consist of a central space surrounded by a layer of columnar
or cuboidal epithelial cells. The nuclei of these cells tend to
be polarized away from the central space. The mechanism
of formation of these tubular structures is not entirely clear
but is likely the result of the secretory activity of the tumor
cells, which appear to be preameloblasts. In any event, these
structures are not true ducts, and no glandular elements are
present in the tumor (Fig. 15-89).
Small foci of calcification may also be scattered through-
out the tumor. These have been interpreted as abortive
enamel formation. Some adenomatoid odontogenic tumors
contain larger areas of matrix material or calcification. This
material has been interpreted as dentinoid or cementum.
Some lesions also have another pattern, particularly at the
periphery of the tumor adjacent to the capsule. This consists
of narrow, often anastomosing cords of epithelium in an
eosinophilic, loosely arranged matrix.
The histopathologic features of this lesion are distinctive
and should not be confused with any other odontogenic
• Fig. 15-88  Adenomatoid Odontogenic Tumor. A well-
circumscribed cystlike mass can be seen enveloping the crown of a
maxillary cuspid. Note the intraluminal vegetations, which represent
nodular tumor growth.
666 C H A P T E R 1 5 Odontogenic Cysts and Tumors
B
• Fig. 15-89  Adenomatoid Odontogenic Tumor. A, Low-power
view demonstrating a thick capsule surrounding the tumor. B, Higher
magnification showing the ductlike epithelial structures. The nuclei of
the columnar cells are polarized away from the central spaces.
tumor. Interestingly, some adenomatoid odontogenic
tumors have been described with focal areas that resemble
calcifying epithelial odontogenic tumor, odontoma, or cal-
cifying odontogenic cyst. These lesions appear to behave as
a routine adenomatoid odontogenic tumor, however. The
chief problem relates to mistaking this tumor for an amelo-
blastoma by a pathologist who is not familiar with this
lesion. This error can lead to unnecessary radical surgery.
Treatment and Prognosis
The adenomatoid odontogenic tumor is completely benign;
because of its capsule, it enucleates easily from the bone.
Aggressive behavior has not been documented, and recur-
rence after enucleation seldom, if ever, occurs.
◆ CALCIFYING EPITHELIAL
ODONTOGENIC TUMOR
(PINDBORG TUMOR)
The calcifying epithelial odontogenic tumor, also widely
known as the Pindborg tumor, is an uncommon lesion that
accounts for less than 1% of all odontogenic tumors.
Approximately 200 cases have been reported to date.
21% 8%
57% 14%
• Fig. 15-90  Calcifying Epithelial Odontogenic Tumor. Relative
distribution of calcifying epithelial odontogenic tumor in the jaws.
• Fig. 15-91  Calcifying Epithelial Odontogenic Tumor. Honey-
combed multilocular radiolucency containing fine calcifications.
Although the tumor is clearly of odontogenic origin, its
histogenesis is uncertain. The tumor cells bear a close mor-
phologic resemblance to the cells of the stratum interme-
dium of the enamel organ; however, some investigators have
recently suggested that the tumor arises from dental lamina
remnants based on its anatomic distribution in the jaws.
Mutations of the PTCH1 gene have been identified in one
small series of this neoplasm. This gene is characteristically
associated with nevoid basal cell carcinoma syndrome (see
page 640), but the calcifying epithelial odontogenic tumor
is not a component of that condition.
Clinical and Radiographic Features
Although the calcifying epithelial odontogenic tumor has
been found in patients across a wide age range and in many
parts of the jaw, it is most often encountered in patients
between 30 and 50 years of age. There is no sex predilection.
About two-thirds of all reported cases have been found in
the mandible, most often in the posterior areas (Fig. 15-90).
A painless, slow-growing swelling is the most common pre-
senting sign.
Radiographically, the tumor exhibits either a unilocular
or a multilocular radiolucent defect (Fig. 15-91), with the
 CHAPTER 15  Odontogenic Cysts and Tumors 667

unilocular pattern encountered more commonly in the Several microscopic variations may be encountered.
maxilla. The margins of the lytic defect are often scalloped Some tumors consist of large sheets of epithelial cells with
and usually relatively well defined. However, approximately minimal production of amyloid-like material and calcifica-
20% of cases have an ill-defined periphery, and an addi- tions. Others show large diffuse masses of amyloid-like
tional 20% exhibit a corticated border. The tumor is material that contain only small nests or islands of epithe-
frequently associated with an impacted tooth, most often lium. A clear cell variant has been described, in which
a mandibular molar. The lesion may be entirely radiolu- clear cells constitute a significant portion of the epithelial
cent, but calcified structures of varying size and density
are commonly seen. Although some authors have suggested
that these are often most prominent around the crown
of the impacted tooth (Fig. 15-92), a review of the lit-
erature identified this feature in only 12% of published
cases with adequate radiographic documentation. Similarly,
the description of a “driven-snow” pattern of the calcifica-
tions appears to be much less common than previously
believed.
A few cases of peripheral (extraosseous) calcifying epithe-
lial odontogenic tumor have been reported. These appear as
nonspecific, sessile gingival masses, most often on the ante-
rior gingiva. Some of these have been associated with
cupped-out erosion of the underlying bone.
A
Histopathologic Features
The calcifying epithelial odontogenic tumor has discrete
islands, strands, or sheets of polyhedral epithelial cells in a
fibrous stroma (Fig. 15-93). The cellular outlines of the
epithelial cells are distinct, and intercellular bridges may be
noted. The nuclei show considerable variation, and giant
nuclei may be seen. Some tumors show considerable nuclear
pleomorphism, but this feature is not considered to indicate
malignancy. Large areas of amorphous, eosinophilic, hyalin-
ized (amyloid-like) extracellular material are also often
present. The tumor islands frequently enclose masses of this
hyaline material, resulting in a cribriform appearance. Cal- B
cifications, which are a distinctive feature of the tumor,
develop within the amyloid-like material and form concen- • Fig. 15-93  Calcifying Epithelial Odontogenic Tumor. A, Sheets
tric rings (Liesegang ring calcifications) (Fig. 15-94). These of epithelial tumor cells that surround pools of amorphous, eosinophilic
amyloid with focal calcification. B, Higher-power view showing poly-
tend to fuse and form large, complex masses. hedral cells with eosinophilic cytoplasm and intercellular bridging. Adja-
cent amyloid deposits can be seen.

• Fig. 15-92  Calcifying Epithelial Odontogenic Tumor. Prominent

calcification around the crown of an impacted second molar that is • Fig. 15-94  Calcifying Epithelial Odontogenic Tumor. Multiple
involved in the tumor. (Courtesy of Dr. Harold Peacock.) concentric Liesegang ring calcifications.
668 C H A P T E R 1 5 Odontogenic Cysts and Tumors
• Fig. 15-95  Calcifying Epithelial Odontogenic Tumor. With
Congo red staining, the pools of amyloid exhibit an apple-green bire-
fringence when viewed with polarized light.
component, and this tumor also has been reported to have
a cystic growth pattern.
The amyloid-like material in the Pindborg tumor has
been extensively investigated by histochemical, immunohis-
tochemical, and biochemical methods, as well as by electron
microscopy. The material generally stains as amyloid (i.e.,
positive staining results with Congo red). After Congo red
staining, the amyloid will exhibit apple-green birefringence
when viewed with polarized light (Fig. 15-95). Investigators
have identified this material as a unique protein that is
produced by this tumor, as well as by the normal odonto-
genic apparatus and other odontogenic neoplasms. Both the
protein structure and the DNA sequence of the responsible
gene have been described, and this material has been
designated as odontogenic ameloblast-associated protein
(ODAM).
Treatment and Prognosis
Although it was originally believed that the calcifying epi-
thelial odontogenic tumor had about the same biologic
behavior as the ameloblastoma, accumulating experience
indicates that it tends to be less aggressive. Conservative
local resection to include a narrow rim of surrounding bone
appears to be the treatment of choice, although lesions in
the posterior maxilla should probably be treated more
aggressively. A recurrence rate of about 15% has been
reported; tumors treated by curettage have the highest fre-
quency of recurrence. The overall prognosis appears good,
although rare examples of malignant or borderline malig-
nant calcifying epithelial odontogenic tumor have been
reported, with documented metastasis to regional lymph
nodes and lung.
◆ SQUAMOUS ODONTOGENIC TUMOR
Squamous odontogenic tumor is a rare benign odonto-
genic neoplasm that was first described in 1975 and is now
recognized as a distinct entity. Approximately 50 examples
have been reported to date. Most of these have been located
• Fig. 15-96  Squamous Odontogenic Tumor. Lucent defect
extending along the roots of the lateral incisor and first premolar teeth.
(Courtesy of Dr. Ed McGaha.)
within bone, although a few peripheral examples have been
described. Before 1975, this lesion was probably believed to
represent an atypical acanthomatous ameloblastoma or even
a squamous cell carcinoma. The squamous odontogenic
tumor may arise from neoplastic transformation of dental
lamina rests or perhaps the epithelial rests of Malassez. In
some cases, the tumor appears to originate within the peri-
odontal ligament that is associated with the lateral root
surface of an erupted tooth.
Clinical and Radiographic Features
Squamous odontogenic tumors have been found in patients
whose ages ranged from 8 to 74 years (average age, 38).
They are randomly distributed throughout the alveolar pro-
cesses of the maxilla and mandible, with no site of predilec-
tion. A few patients have had multiple squamous odontogenic
tumors that involved several quadrants of the mouth; one
family with three affected siblings who each had multiple
lesions has been reported. There is no apparent sex predilec-
tion. A painless or mildly painful gingival swelling, often
associated with mobility of the associated teeth, is the most
common complaint. About 25% of reported patients have
had no symptoms, and their lesions were detected during a
radiographic examination.
The radiographic findings are not specific or diagnostic
and consist of a triangular radiolucent defect lateral to the
root or roots of the teeth (Fig. 15-96). In some instances,
this suggests vertical periodontal bone loss. The radiolucent
area may be somewhat ill defined or may show a well-
defined, corticated margin. Most examples are relatively
small lesions that seldom exceed 1.5 cm in greatest
diameter.
Histopathologic Features
The microscopic findings of squamous odontogenic tumor
are distinctive and consist of varying-shaped islands of
bland-appearing squamous epithelium in a mature fibrous
connective tissue stroma. The peripheral cells of the

epithelial islands do not show the characteristic polarization
seen in ameloblastomas (Fig. 15-97). Vacuolization and
individual cell keratinization within the epithelial islands
are common features. Small microcysts are sometimes
observed within the epithelial islands. Laminated calcified
bodies and globular eosinophilic structures, which do not
stain for amyloid, are present within the epithelium in some
cases. The former probably represents dystrophic calcifica-
tions; the nature of the latter is unknown.
Islands of epithelium that closely resemble those of the
squamous odontogenic tumor have been observed within
the fibrous walls of dentigerous and radicular cysts. These
have been designated as squamous odontogenic tumorlike pro-
liferations in odontogenic cysts. These islands do not appear
to have any significance relative to the behavior of the cyst,
and evaluation of the clinical, radiographic, and histopatho-
logic features should permit differentiation from a squa-
mous odontogenic tumor.
In published reports, some squamous odontogenic
tumors have been misdiagnosed initially as ameloblastomas,
resulting in unnecessary radical surgery.
Treatment and Prognosis
Conservative local excision or curettage appears to be effec-
tive for patients with squamous odontogenic tumors, and
A
B a rare phenomenon.
• Fig. 15-97  Squamous Odontogenic Tumor. A, Low-power pho-
tomicrograph showing islands of bland-appearing squamous epithe-
lium in a fibrous stroma. B, Higher-power photomicrograph showing
bland appearance of the epithelium with microcyst formation.
CHAPTER 15  Odontogenic Cysts and Tumors 669
most reported cases have not recurred after local excision.
A few instances of recurrence have been reported, but these
have responded well to further local excision. Maxillary
squamous odontogenic tumors may be somewhat more
aggressive than mandibular lesions, with a greater tendency
to invade adjacent structures. This may be because of the
porous, spongy nature of the maxillary bone. The multicen-
tric lesions have typically exhibited a less aggressive, almost
hamartomatous behavior when compared with solitary
lesions. A well-documented example of apparent malignant
transformation of squamous odontogenic tumor has been
reported.
MIXED ODONTOGENIC TUMORS
The group of mixed odontogenic tumors, composed of
proliferating odontogenic epithelium in a cellular ectomes-
enchyme resembling the dental papilla, poses problems in
classification. Some of these lesions show varying degrees of
inductive effect by the epithelium on the mesenchyme,
leading to the formation of varying amounts of enamel and
dentin. Some of these lesions (the common odontomas)
are clearly nonneoplastic developmental anomalies; others
appear to be true neoplasms. The nature of others is
uncertain.
In some instances, the histopathologic findings alone
cannot distinguish between the neoplastic lesions and the
developmental anomalies. Clinical and radiographic fea-
tures often are of considerable assistance in making this
distinction.
◆ AMELOBLASTIC FIBROMA
The ameloblastic fibroma is considered to be a true mixed
tumor in which the epithelial and mesenchymal tissues are
both neoplastic. It is an uncommon tumor, but the data
regarding its frequency are difficult to evaluate because (par-
ticularly in earlier reports) some lesions that were diagnosed
as ameloblastic fibroma may actually have represented the
early developing stage of an odontoma.
Clinical and Radiographic Features
Ameloblastic fibromas tend to occur in younger patients;
most lesions are diagnosed in the first two decades of life.
This lesion, however, is occasionally encountered in middle-
aged patients. The tumor is slightly more common in males
than in females. Small ameloblastic fibromas are asymptom-
atic; larger tumors are associated with swelling of the jaws.
The posterior mandible is the most common site; about
70% of all cases are located in this area (Fig. 15-98). Con-
vincing examples of this tumor arising within the gingival
soft tissue have been described, but this appears to represent
Radiographically, either a unilocular or multilocular
radiolucent lesion is seen, with the smaller lesions tending
to be unilocular. The radiographic margins tend to be well
defined, and they may be corticated. An unerupted tooth
670 C H A P T E R 1 5 Odontogenic Cysts and Tumors
23% 4%
69% 4%
• Fig. 15-98  Ameloblastic Fibroma. Relative distribution of amelo-
blastic fibroma in the jaws.
• Fig. 15-99  Ameloblastic Fibroma. Multilocular radiolucent defect
associated with an unerupted second molar. (Courtesy of Dr. Mark
Chishom.)
is associated with the lesion in about 75% of cases (Fig.
15-99). The ameloblastic fibroma may grow to a large size,
and cases that involve a considerable portion of the body
and ascending ramus of the mandible have been reported.
Histopathologic Features
The ameloblastic fibroma appears as a solid, soft tissue mass
with a smooth outer surface. A definite capsule may or may
not be present. Microscopically, the tumor is composed of
a cell-rich mesenchymal tissue resembling the primitive
dental papilla admixed with proliferating odontogenic epi-
thelium. The latter may have one of two patterns, both of
which are usually present in any given case. The most
common epithelial pattern consists of long, narrow cords of
odontogenic epithelium, often in an anastomosing arrange-
ment. These cords are usually only two cells in thickness
and are composed of cuboidal or columnar cells (Fig.
15-100). In the other pattern, the epithelial cells form small,
discrete islands that resemble the follicular stage of the
developing enamel organ. These show peripheral columnar
• Fig. 15-100  Ameloblastic Fibroma. A, Long, narrow cords of
odontogenic epithelium supported by richly cellular, primitive connec-
tive tissue. B, Basophilic epithelial islands with peripheral nuclear
palisading.
cells, which surround a mass of loosely arranged epithelial
cells that resemble stellate reticulum. In contrast to the fol-
licular type of ameloblastoma, these follicular islands in
the ameloblastic fibroma seldom demonstrate microcyst
formation.
The mesenchymal portion of the ameloblastic fibroma
consists of plump stellate and ovoid cells in a loose matrix,
which closely resembles the developing dental papilla. Col-
lagen formation is generally inconspicuous. Juxtaepithelial
hyalinization of the mesenchymal portion of the tumor is
sometimes seen, and occasionally diffuse areas of hyalinized
acellular lesional tissue are evident.
A few examples of ameloblastic fibroma occurring in
conjunction with calcifying odontogenic cyst also have been
reported.
Treatment and Prognosis
The proper management of ameloblastic fibroma has been
an ongoing topic of debate. Although initially it was believed
that the ameloblastic fibroma was an innocuous lesion that
seldom recurred after simple local excision or curettage,
subsequent reports seemed to indicate a substantial risk of
recurrence after conservative therapy. The highest recur-
rence rate (43.5%) was recorded in a series of cases from

the Armed Forces Institute of Pathology, and it could be
argued that this was a biased sample of larger lesions that
were inherently more difficult to manage. In other series of
cases, from 0% to 18% of ameloblastic fibromas were
reported to recur after conservative removal and an ade-
quate follow-up period. Based on these data, recent recom-
mendations have emphasized conservative initial therapy
for ameloblastic fibroma. More aggressive surgical excision
should probably be reserved for recurrent lesions. Approxi-
mately 35% of the cases of the rare ameloblastic fibrosar-
coma develop in the setting of a recurrent ameloblastic
fibroma.
◆ AMELOBLASTIC FIBRO-ODONTOMA
The ameloblastic fibro-odontoma is defined as a tumor
with the general features of an ameloblastic fibroma but
that also contains enamel and dentin. Some investigators
believe that the ameloblastic fibro-odontoma is only a
stage in the development of an odontoma and do not
consider it to be a separate entity. Certainly the histo-
pathologic features of a developing odontoma may overlap
somewhat with ameloblastic fibro-odontoma. There are
well-documented examples, however, of this tumor exhibit-
ing progressive growth and causing considerable deformity
and bone destruction. Such lesions appear to be true
neoplasms. However, distinguishing between a developing
odontoma and an ameloblastic fibro-odontoma may be
difficult based on histopathologic grounds alone.
Clinical and Radiographic Features
The ameloblastic fibro-odontoma is usually seen in children
with an average age of 10 years. It is rarely encountered in
adults. Like the ameloblastic fibroma, ameloblastic fibro-
odontomas occur more frequently in the posterior regions
• Fig. 15-102  Ameloblastic Fibro-Odontoma. Radiolucent defect in the ramus containing small
calcifications having the radiodensity of tooth structure.
CHAPTER 15  Odontogenic Cysts and Tumors 671
of the jaws, and the majority involves the mandible
(Fig. 15-101). Males are affected somewhat more often than
females, with a 3 : 2 ratio noted in the literature. The lesion
is commonly asymptomatic and is discovered when radio-
graphs are taken to determine the reason for failure of a
tooth to erupt. Large examples may be associated with a
painless swelling of the affected bone.
Radiographically, the tumor shows a well-circumscribed
unilocular or, infrequently, multilocular radiolucent defect
that contains a variable amount of calcified material with
the radiodensity of tooth structure. The calcified material
within the lesion may appear as multiple, small radiopacities
or as a solid conglomerate mass (Fig. 15-102). In most
instances, an unerupted tooth is present at the margin of
the lesion, or the crown of the unerupted tooth may be
included within the defect. Approximately 5% of amelo-
blastic fibro-odontomas contain only a minimal amount
of calcifying enamel and dentin matrix and appear
21% 14%
54% 11%
• Fig. 15-101  Ameloblastic Fibro-Odontoma. Relative distribution
of ameloblastic fibro-odontoma in the jaws.
672 C H A P T E R 1 5 Odontogenic Cysts and Tumors

• Fig. 15-103  Ameloblastic Fibro-Odontoma. Unilocular radiolu- A

cent defect displacing the developing mandibular third molar posteri-
orly. Flecks of mineralized material are present in the radiolucent
defect. (Courtesy of Dr. Dominic Adornato.)

radiographically as radiolucent lesions (Fig. 15-103). These

cannot be differentiated from the wide variety of unilocular
radiolucencies that may involve the jaws. At the other
extreme, some ameloblastic fibro-odontomas appear as
largely calcified masses with only a narrow rim of radiolu-
cency about the periphery of the lesion.

Histopathologic Features
The soft tissue component of the ameloblastic fibro-
odontoma is microscopically identical to the ameloblastic
fibroma and has narrow cords and small islands of odon-
togenic epithelium in a loose primitive-appearing connec-
tive tissue that resembles the dental papilla. The calcifying
element consists of foci of enamel and dentin matrix forma-
tion in close relationship to the epithelial structures (Fig.
15-104). The more calcified lesions show mature dental
structures in the form of rudimentary small teeth or con-
glomerate masses of enamel and dentin. Some researchers
have designated a similar tumor in which the calcifying
component consists only of dentin matrix and dentinoid
material as ameloblastic fibro-dentinoma. It is question-
able whether this lesion represents a separate entity, and it
is probably best considered as only a variant of the amelo-
blastic fibro-odontoma.
Treatment and Prognosis
A patient with an ameloblastic fibro-odontoma is generally
treated by conservative curettage, and the lesion usually
separates easily from its bony bed. The tumor is well cir-
cumscribed and does not invade the surrounding bone.
The prognosis is excellent, and the recurrence rate after
conservative removal is estimated to be about 7%. Develop-
ment of an ameloblastic fibrosarcoma after curettage of an
ameloblastic fibro-odontoma has been reported, but this is
exceedingly rare.
B
• Fig. 15-104  Ameloblastic Fibro-Odontoma. A, The soft tissue
component of the tumor is indistinguishable from an ameloblastic
fibroma. B, Formation of disorganized tooth structure can be seen.
◆ AMELOBLASTIC FIBROSARCOMA
(AMELOBLASTIC SARCOMA)
The rare ameloblastic fibrosarcoma is considered to be the
malignant counterpart of the ameloblastic fibroma, and
approximately 70 cases have been documented in the litera-
ture. Interestingly, in most cases, only the mesenchymal
portion of the lesion shows features of malignancy; the
epithelial component remains rather bland. These tumors
may apparently arise de novo; however, in at least one-third
of known cases, the malignant lesion represents a recurrence
of a tumor previously diagnosed as an ameloblastic fibroma
or an ameloblastic fibro-odontoma.
Clinical and Radiographic Features
Ameloblastic fibrosarcomas occur about 1.5 times as often
in males as in females. The lesion tends to occur in younger
patients (mean reported age, 27.5 years). Although either the
maxilla or the mandible may be involved, about 80% of cases
have occurred in the mandible. Pain and swelling associated
with rapid clinical growth are the common complaints.
 CHAPTER 15  Odontogenic Cysts and Tumors 673

A B
• Fig. 15-105  Ameloblastic Fibrosarcoma. A, A 21-year-old woman complained of facial asymmetry
and recent increase in size of a mandibular mass that had been present for some years. B, Radiograph
of the same patient. Note the lytic destruction of the posterior mandible. (Courtesy of Dr. Sam McKenna.)

Radiographically, the ameloblastic fibrosarcoma shows

an ill-defined destructive radiolucent lesion that suggests a
malignant process (Fig. 15-105).

Histopathologic Features
Ameloblastic fibrosarcomas contain an epithelial compo-
nent similar to that seen in the ameloblastic fibroma,
although it is frequently less prominent. The epithelial com-
ponent appears histopathologically benign and does not
demonstrate any cytologic atypia. The mesenchymal portion
of the tumor, however, is highly cellular and shows hyper-
chromatic and often bizarre pleomorphic cells (Fig. 15-106).
Mitoses are usually prominent. In some cases with multiple
recurrences, the epithelial component becomes progres-
sively less conspicuous so that the tumor eventually shows
only a poorly differentiated fibrosarcoma.
In a few instances, dysplastic dentin or small amounts
of enamel may be formed. Some have called such lesions
ameloblastic dentinosarcomas or ameloblastic fibro-
odontosarcomas. This additional subclassification, however,
appears unnecessary. Another rare event that actually may
be overrepresented in the literature is concurrent malignant
transformation of both the epithelial and mesenchymal ele-
ments of an ameloblastic fibroma, resulting in an amelo-
blastic carcinosarcoma.
Treatment and Prognosis
Once the diagnosis of ameloblastic fibrosarcoma has been
confirmed, radical surgical excision appears to be the treat-
ment of choice. Curettage or local excision is usually fol-
lowed by rapid local recurrence. The tumor is locally
aggressive and infiltrates adjacent bone and soft tissues.
The long-term prognosis is difficult to ascertain because
of the few reported cases with adequate follow-up, with the
best estimates suggesting that 20% of these patients will
succumb to their tumor. Most deaths have resulted from
uncontrolled local disease, and metastatic tumor has been
documented in only four of 54 evaluable cases.
• Fig. 15-106  Ameloblastic Fibrosarcoma. The cellular mesen-
chymal tissue shows hyperchromatism and atypical cells. A small
island of ameloblastic epithelium is present.
◆ ODONTOAMELOBLASTOMA
The odontoameloblastoma is an extremely rare odonto-
genic tumor that contains an ameloblastomatous compo-
nent and odontoma-like elements. Fewer than 20 cases have
been reported with sufficient documentation to support this
diagnosis. This tumor was formerly called ameloblastic odon-
toma and was confused with the more common (though
still relatively rare) lesion currently designated as amelo-
blastic fibro-odontoma. Because the clinical behavior of
these two tumors is quite different, they should be distin-
guished from one another. This neoplasm is also frequently
confused with an odontoma that is in its early stages of
development.
Clinical and Radiographic Features
Because of the rarity of odontoameloblastomas, little reli-
able information is available. The lesion appears to occur
more often in younger patients, and either jaw can be
affected. Pain, delayed eruption of teeth, and expansion of
the affected bone may be noted.
674 C H A P T E R 1 5 Odontogenic Cysts and Tumors

Radiographically, the tumor shows a radiolucent, destruc-
tive process that contains calcified structures. These have the
radiodensity of tooth structure and may resemble miniature
teeth or occur as larger masses of calcified material similar
to a complex odontoma.
Histopathologic Features
The histopathologic features of the odontoameloblastoma
are complex. The proliferating epithelial portion of the
tumor has features of an ameloblastoma, most often of the
plexiform or follicular pattern. The ameloblastic component
is intermingled with immature or more mature dental tissue
in the form of developing rudimentary teeth, which is
similar to the appearance of a compound odontoma, or
conglomerate masses of enamel, dentin, and cementum, as
seen in a complex odontoma.
a tooth to erupt. Odontomas are typically relatively small
and seldom exceed the size of a tooth in the area where they
are located. However, large odontomas up to 6 cm or more
in diameter are occasionally seen. These large odontomas
can cause expansion of the jaw.
Odontomas occur somewhat more frequently in the
maxilla than in the mandible. Although compound and
complex odontomas may be found in any site, the com-
pound type is more often seen in the anterior maxilla;
complex odontomas occur more often in the molar regions
of either jaw. Occasionally, an odontoma will develop com-
pletely within the gingival soft tissues.
Radiographically, the compound odontoma appears as
a collection of toothlike structures of varying size and shape
surrounded by a narrow radiolucent zone (Figs. 15-107 and
15-108). The complex odontoma appears as a calcified

Treatment and Prognosis

Multiple recurrences of odontoameloblastomas have been
reported after local curettage, and it appears that this tumor
has the same biologic potential as the ameloblastoma. It is
probably wise to treat a patient with this lesion in the same
manner as one with an ameloblastoma. However, there are
no valid data on the long-term prognosis.

◆ ODONTOMA
Odontomas are the most common types of odontogenic
tumors. Their prevalence exceeds that of all other odonto-
genic tumors combined. Odontomas are considered to be • Fig. 15-107  Compound Odontoma. A small cluster of toothlike
structures is preventing the eruption of the maxillary canine. (Courtesy
developmental anomalies (hamartomas), rather than true
of Dr. Robert J. Powers.)
neoplasms. When fully developed, odontomas consist
chiefly of enamel and dentin, with variable amounts of pulp
and cementum. In their earlier developmental stages,
varying amounts of proliferating odontogenic epithelium
and mesenchyme are present.
Odontomas are further subdivided into compound and
complex types. The compound odontoma is composed of
multiple, small toothlike structures. The complex odon-
toma consists of a conglomerate mass of enamel and dentin,
which bears no anatomic resemblance to a tooth. In most
series, compound odontomas are more frequently diag-
nosed than complex, and it is possible that some compound
odontomas are not submitted for microscopic examination
because the clinician is comfortable with the clinical and
radiographic diagnosis. Occasionally, an odontoma may
show both compound and complex features.

Clinical and Radiographic Features

Most odontomas are detected during the first two decades
of life, and the mean age at the time of diagnosis is 14 years.
The majority of these lesions are completely asymptomatic,
being discovered on a routine radiographic examination or • Fig. 15-108  Compound Odontoma. Multiple toothlets preventing
when films are taken to determine the reason for failure of the eruption of the mandibular cuspid. (Courtesy of Dr. Brent Bernard.)

• Fig. 15-109  Complex Odontoma. A large radiopaque mass is overlying the crown of the mandibular
right second molar, which has been displaced to the inferior border of the mandible.
• Fig. 15-110  Compound Odontoma. Surgical specimen consist-
ing of more than 20 malformed toothlike structures.
mass with the radiodensity of tooth structure, which is also
surrounded by a narrow radiolucent rim. An unerupted
tooth is frequently associated with the odontoma, and the
odontoma prevents eruption of the tooth (Fig. 15-109).
Some small odontomas are present between the roots of
erupted teeth and are not associated with disturbance in
eruption. The radiographic findings are usually diagnostic,
and the compound odontoma is seldom confused with any
other lesion. A developing odontoma may show little evi-
dence of calcification and appear as a circumscribed radio-
lucent lesion. A complex odontoma, however, may be
radiographically confused with an osteoma or some other
highly calcified bone lesion.
Histopathologic Features
The compound odontoma consists of multiple structures
resembling small, single-rooted teeth, contained in a loose
fibrous matrix (Fig. 15-110). The mature enamel caps of
the toothlike structures are lost during decalcification for
preparation of the microscopic section, but varying amounts
of enamel matrix are often present. Pulp tissue may be seen
CHAPTER 15  Odontogenic Cysts and Tumors 675
• Fig. 15-111  Complex Odontoma. This decalcified section shows
a disorganized mass of dentin intermixed with small pools of enamel
matrix.
in the coronal and root portions of the toothlike structures.
In patients with developing odontomas, structures that
resemble tooth germs are present.
Complex odontomas consist largely of mature tubular
dentin. This dentin encloses clefts or hollow circular struc-
tures that contained the mature enamel that was removed
during decalcification. The spaces may contain small
amounts of enamel matrix or immature enamel (Fig.
15-111). Small islands of eosinophilic-staining epithelial
ghost cells are present in about 20% of complex odontomas.
These may represent remnants of odontogenic epithelium
that have undergone keratinization and cell death from the
local anoxia. A thin layer of cementum is often present
about the periphery of the mass. Occasionally, a dentigerous
cyst may arise from the epithelial lining of the fibrous
capsule of a complex odontoma.
Treatment and Prognosis
Odontomas are treated by simple local excision, and the
prognosis is excellent.
676 C H A P T E R 1 5 Odontogenic Cysts and Tumors

the teeth often cause root divergence. Approximately 12%

of central odontogenic fibromas will exhibit radiopaque
TUMORS OF ODONTOGENIC flecks within the lesion.
ECTOMESENCHYME
Histopathologic Features
◆ CENTRAL ODONTOGENIC FIBROMA
Lesions reported as central odontogenic fibroma have shown
The central odontogenic fibroma is an uncommon and considerable histopathologic diversity; this has led some
somewhat controversial lesion. Approximately 100 exam- authors to describe two separate types, although this concept
ples have been reported. Formerly, some oral and maxillo- has been questioned. The World Health Organization
facial pathologists designated solid fibrous masses that were (WHO) recognizes an epithelium-poor variant of central
almost always associated with the crown of an unerupted
tooth as odontogenic fibromas. Most oral and maxillofacial
pathologists today consider such lesions to represent only
hyperplastic dental follicles, and these should not be con-
sidered to be neoplasms.

Clinical and Radiographic Features

Odontogenic fibromas have been reported in patients whose
ages ranged from 4 to 80 years (mean age, 40 years). Of
those cases reported in the literature, a 1.8 : 1.0 female-to-
male ratio has been noted, indicating a strong female pre-
dilection. The maxilla and mandible are affected nearly
equally, with most maxillary lesions located anterior to the
first molar tooth (Fig. 15-112). In the mandible, however, A
about half of the tumors are located posterior to the first
molar. One-third of odontogenic fibromas are associated
with an unerupted tooth. Smaller odontogenic fibromas are
usually completely asymptomatic; larger lesions may be
associated with localized bony expansion or loosening of
teeth. Interestingly, the palatal mucosa that overlies the
tumor occasionally may exhibit a defect or groove.
Radiographically, smaller odontogenic fibromas tend to
be well-defined, unilocular, radiolucent lesions often associ-
ated with the periradicular area of erupted teeth (Fig.
15-113). Larger lesions tend to be multilocular radiolucen-
cies. Many lesions have a corticated border. Root resorption
of associated teeth is common, and lesions located between

10% 6% 29%

29% 18% 8%
B
• Fig. 15-113  Odontogenic Fibroma. A, Clinical image showing a
groove or defect in the palatal mucosa, a feature that has been
described with maxillary lesions. B, Radiograph of this patient, depict-
• Fig. 15-112  Odontogenic Fibroma. Relative distribution of odon- ing a multilocular radiolucency of the anterior maxilla. (Courtesy of 
togenic fibroma in the jaws. Dr. Greg Adams.)

odontogenic fibroma (so-called simple odontogenic
fibroma) and an epithelium-rich variant (so-called WHO
odontogenic fibroma). The simple type of odontogenic
fibroma is composed of stellate fibroblasts, often arranged
in a whorled pattern with fine collagen fibrils and consider-
able ground substance (Fig. 15-114). Small foci of odonto-
genic epithelial rests should be present according to the
WHO definition. Spindle cell collagenous lesions that do
not have epithelial rests may represent other entities, such
as desmoplastic fibroma, myofibroma, or neurofibroma.
Occasional foci of dystrophic calcification may be seen.
The epithelium-rich odontogenic fibroma has a more
complex pattern, which often consists of a fairly cellular
fibrous connective tissue with collagen fibers arranged in
interlacing bundles. Odontogenic epithelium in the form
of long strands or isolated nests is present throughout the
lesion and may be a prominent component (Fig. 15-115).
The fibrous component may vary from myxoid to densely
hyalinized. Calcifications composed of cementum-like
• Fig. 15-114  Odontogenic Fibroma (Simple Type). Scattered
fibroblasts within a collagenous background. No epithelial rests were
found on multiple sections from this tumor.
• Fig. 15-115  Odontogenic Fibroma (World Health Organiza-
tion [WHO] Type). A cellular fibroblastic lesion containing narrow
cords of odontogenic epithelium.
CHAPTER 15  Odontogenic Cysts and Tumors 677
material or dentinoid are present in some cases. Focal
deposits of odontogenic ameloblast-associated protein
(ODAM), which represent a form of amyloid, have been
described in a few central odontogenic fibromas, and the
possibility that some of these lesions may represent calcify-
ing epithelial odontogenic tumors cannot be excluded.
Approximately 20 examples of central odontogenic fibroma
associated with a giant cell granuloma–like component
have been reported since 1992 (Fig. 15-116). It seems
unlikely that this process represents a “collision” tumor
with synchronous occurrence of an odontogenic fibroma
and a giant cell granuloma. Several of these lesions have
recurred, and the recurrences typically exhibit both com-
ponents. Whether the odontogenic fibroma somehow
induced a giant cell response in these patients, a giant cell
granuloma triggered formation of an odontogenic fibroma,
or whether this is a distinct biphasic lesion remains to be
clarified.
A
B
• Fig. 15-116  Odontogenic Fibroma (WHO Type) with Associ-
ated Giant Cell Granuloma. A, Unilocular radiolucency between the
left mandibular bicuspids. B, Microscopic examination reveals two
distinct patterns. On the left, one can see cords of odontogenic epi-
thelium within a fibrous background, consistent with odontogenic
fibroma (WHO type). Typical features of central giant cell granuloma
are present on the right side of the field.
678 C H A P T E R 1 5 Odontogenic Cysts and Tumors
Treatment and Prognosis
Odontogenic fibromas are usually treated by enucleation
and vigorous curettage. Although the tumor does not have
a definite capsule, it appears to have a limited growth poten-
tial, particularly in the anterior regions of the jaws. A few
recurrences have been documented, but the prognosis is
very good.
◆ PERIPHERAL ODONTOGENIC FIBROMA
The relatively uncommon peripheral odontogenic fibroma
is considered to represent the soft tissue counterpart of the
central (intraosseous) odontogenic fibroma. In the past,
some authors have designated clinically and histopathologi-
cally similar lesions as odontogenic epithelial hamartoma
or as peripheral fibroameloblastic dentinoma. It is likely
that all of these terms refer to the same lesion, and periph-
eral odontogenic fibroma seems to be the most appropriate
designation. A few series of this lesion have been reported
in the past three decades, bringing the total number of cases
in the literature to over 375.
Clinical and Radiographic Features
The peripheral odontogenic fibroma appears as a firm, slow-
growing, and usually sessile gingival mass covered by
normal-appearing mucosa (Fig. 15-117). Rarely, multifocal
or diffuse lesions have been described. Clinically, the
peripheral odontogenic fibroma cannot be distinguished
from the much more common fibrous gingival lesions (see
Chapter 12). The lesion is most often encountered on the
facial gingiva of the mandible. Most lesions are between
0.5 and 1.5  cm in diameter, and they infrequently cause
displacement of the teeth. Peripheral odontogenic fibromas
have been recorded in patients across a wide age range,
with most identified from the second to the fourth decades
of life.
• Fig. 15-117  Peripheral Odontogenic Fibroma. This sessile gin-
gival mass cannot be clinically distinguished from the common periph-
eral ossifying fibroma. (Courtesy of Dr. Jerry Stovall.)
Radiographic studies demonstrate a soft tissue mass,
which in some cases has shown areas of calcification. The
lesion typically does not involve the underlying bone,
although occasionally a “cupped out” appearance has been
noted.
Histopathologic Features
The peripheral odontogenic fibroma shows similar histo-
pathologic features to the central odontogenic fibroma
(WHO type). The tumor consists of interwoven fascicles of
cellular fibrous connective tissue, which may be interspersed
with areas of less cellular, myxoid connective tissue. A gran-
ular cell change has been rarely identified in the connective
tissue component, and giant cell granuloma–like areas have
been described in a few lesions. Islands or strands of odon-
togenic epithelium are scattered throughout the connective
tissue. These may be prominent or scarce. The epithelial
cells may show vacuolization. Dysplastic dentin, amorphous
ovoid cementum-like calcifications, and trabeculae of
osteoid may also be present.
Treatment and Prognosis
The peripheral odontogenic fibroma is treated by local sur-
gical excision, and the prognosis is good. Recurrence of this
lesion has been documented, however, so the patient and
clinician should be aware of this possibility.
◆GRANULAR CELL ODONTOGENIC
TUMOR (GRANULAR CELL
ODONTOGENIC FIBROMA)
The rare granular cell odontogenic tumor was initially
reported as “granular cell ameloblastic fibroma.” Subse-
quently, it was designated as granular cell odontogenic
fibroma, but the noncommittal term granular cell odon-
togenic tumor is probably more appropriate, given the
controversial nature of the lesion. Approximately 30 cases
of this unusual tumor have been reported.
Clinical and Radiographic Features
Patients with granular cell odontogenic tumors have all
been adults at the time of diagnosis, with more than half
being older than 40 years of age. More than 70% of the
cases have developed in women. The tumor occurs pri-
marily in the mandible and most often in the premolar
and molar region. Some lesions are completely asymp-
tomatic; others present as a painless, localized expansion
of the affected area. A few cases of granular cell odonto-
genic tumor have been described in the gingival soft tissues
as well.
Radiographically, the lesion appears as a well-demarcated
radiolucency, which may be unilocular or multilocular and
occasionally shows small calcifications (Fig. 15-118).

• Fig. 15-118  Granular Cell Odontogenic Tumor. Radiolucent
lesion involving the apical area of endodontically treated maxillary
teeth. (Courtesy of Dr. Steve Ferry.)
• Fig. 15-119  Granular Cell Odontogenic Tumor. Sheet of large
granular mesenchymal cells with small nests of odontogenic
epithelium.
Histopathologic Features
The granular cell odontogenic tumor is composed of large
eosinophilic granular cells, which closely resemble the gran-
ular cells seen in the soft tissue granular cell tumor (see page
502) or the granular cells seen in the granular cell variant
of the ameloblastoma (see page 657). Narrow cords or small
islands of odontogenic epithelium are scattered among the
granular cells (Fig. 15-119). Small cementum-like or dys-
trophic calcifications associated with the granular cells have
been seen in some lesions.
The nature of the granular cells is controversial. Ultra-
structural studies reveal the features of mesenchymal cells,
and bodies consistent with lysosomal structures have been
identified within the lesional cell cytoplasm. Immunohisto-
chemically, the granular cells in the granular cell odonto-
genic tumor do not react with antibodies directed against
S-100 protein, in contrast to the positive S-100 reactivity
of the granular cell tumor.
CHAPTER 15  Odontogenic Cysts and Tumors 679
16% 9% 9%
28% 20% 18%
• Fig. 15-120  Odontogenic Myxoma. Relative distribution of odon-
togenic myxoma in the jaws.
Treatment and Prognosis
The granular cell odontogenic fibroma appears to be com-
pletely benign in the overwhelming majority of instances
and responds well to curettage. Only one recurrence has
been documented, and a solitary example of a malignant
central granular cell odontogenic fibroma has been reported.
◆ ODONTOGENIC MYXOMA
Myxomas of the jaws are believed to arise from odontogenic
ectomesenchyme. They bear a close microscopic resem-
blance to the mesenchymal portion of a developing tooth.
Formerly, some investigators made a distinction between
odontogenic myxomas (derived from odontogenic mesen-
chyme) and osteogenic myxomas (presumably derived
from primitive bone tissue). However, most authorities in
orthopedic pathologic practice do not accept that myxomas
occur in the extragnathic skeleton, and all myxomas of the
jaws are currently considered to be of odontogenic origin.
Clinical and Radiographic Features
Myxomas are predominantly found in young adults but
may occur across a wide age group. The average age for
patients with myxomas is 25 to 30 years. There is no sex
predilection. The tumor may be found in almost any area
of the jaws, and the mandible is involved more commonly
than the maxilla (Fig. 15-120). Smaller lesions may be
asymptomatic and are discovered only during a radiographic
examination. Larger lesions are often associated with a pain-
less expansion of the involved bone. In some instances,
clinical growth of the tumor may be rapid; this is probably
related to the accumulation of myxoid ground substance in
the tumor.
Radiographically, the myxoma appears as a unilocular or
multilocular radiolucency that may displace or cause resorp-
tion of teeth in the area of the tumor (Fig. 15-121). The
margins of the radiolucency are often irregular or scalloped.
The radiolucent defect may contain thin, wispy trabeculae
680 C H A P T E R 1 5 Odontogenic Cysts and Tumors

• Fig. 15-123  Odontogenic Myxoma. Multilocular expansile radio-

lucency of the posterior mandible. (Courtesy of Dr. Steve Anderson.)

• Fig. 15-121  Odontogenic Myxoma. Unilocular radiolucency

between the right mandibular lateral incisor and cuspid.

• Fig. 15-124  Odontogenic Myxoma. Gross specimen of case

shown in Fig. 15-121, demonstrating a white gelatinous mass.

• Fig. 15-122  Odontogenic Myxoma. Radiolucent lesion of ante-

rior maxilla showing fine residual bone trabeculae arranged at right
angles to one another (“stepladder” pattern).

of residual bone, which are often arranged at right angles

to one another (Fig. 15-122). Large myxomas of the man-
dible may show a “soap bubble” radiolucent pattern, which
is indistinguishable from that seen in ameloblastomas (Fig.
15-123).
• Fig. 15-125  Odontogenic Myxoma. A loose, myxomatous tumor
can be seen filling the marrow spaces between the bony trabeculae.
Histopathologic Features The inset shows stellate-shaped cells and fine collagen fibrils.

At the time of surgery or gross examination of the specimen,

the gelatinous, loose structure of the myxoma is obvious
(Fig. 15-124). Microscopically, the tumor is composed of
haphazardly arranged stellate, spindle-shaped, and round
cells in an abundant, loose myxoid stroma that contains
only a few collagen fibrils (Fig. 15-125). Histochemical
study shows that the ground substance is composed of
glycosaminoglycans, chiefly hyaluronic acid and chondroi-
tin sulfate. Immunohistochemically, the myxoma cells show
diffuse immunoreactivity with antibodies directed against
vimentin, with focal reactivity for muscle-specific actin.

Small islands of inactive-appearing odontogenic epithelial
rests may be scattered throughout the myxoid ground sub-
stance. These epithelial rests are not required for the diag-
nosis and are not obvious in most cases. In some patients,
the tumor may have a greater tendency to form collagen
fibers; such lesions are sometimes designated as fibromyxo-
mas or myxofibromas. There is no evidence that the more
collagenized variants deserve separate consideration,
although some investigators have suggested that these may
represent part of a spectrum that includes the central odon-
togenic fibroma at the other endpoint. Myxomas may rarely
exhibit cementum-like calcifications.
A myxoma may be microscopically confused with other
myxoid jaw neoplasms, such as the rare chondromyxoid
fibroma (see page 611) or the myxoid neurofibroma (see
page 494). Chondromyxoid fibroma should have areas of
cartilaginous differentiation, whereas myxoid neurofibro-
mas tend to have areas in which lesional cells are arranged in
vague fascicles, as well as scattered cells that are positive for
antibodies directed against S-100 protein. Myxoid change
in an enlarged dental follicle or the dental papilla of a devel-
oping tooth may be microscopically similar to a myxoma.
Evaluation of the clinical and radiographic features, however,
prevents overdiagnosis of these lesions as myxomas.
Treatment and Prognosis
Small myxomas are generally treated by curettage, but
careful periodic reevaluation is necessary for at least 5 years.
For larger lesions, more extensive resection may be required
because myxomas are not encapsulated and tend to infiltrate
the surrounding bone. Complete removal of a large tumor
by curettage is often difficult to accomplish, and lesions of
the posterior maxilla, in particular, should be treated more
aggressively in most instances. Recurrence rates from various
studies average approximately 25%. In spite of local recur-
rences, the overall prognosis is good, and metastases do not
occur.
In rare cases the myxoma microscopically shows marked
cellularity and cellular atypism. Some have designated these
lesions as myxosarcomas or malignant odontogenic myxoma.
They appear to have a more aggressive local course than do
the usual myxomas. Death because of involvement of vital
structures by the tumor has been described, but distant
metastases have not been reported.
◆ CEMENTOBLASTOMA
(“TRUE CEMENTOMA”)
Many oral and maxillofacial pathologists consider the
cementoblastoma to represent an odontogenic tumor.
However, other pathologists have pointed out that the his-
topathologic features of cementoblastomas of the jaws are
identical to those of a bone tumor, osteoblastoma, seen both
in the jaws and extragnathic skeleton. Cementoblastomas
are discussed in Chapter 14 (see page 610).
CHAPTER 15  Odontogenic Cysts and Tumors 681
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