CHAPTER 15  Odontogenic Cysts and Tumors 669
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.
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.