CHAPTER 4
Cysts and Tumors of
Odontogenic Origin
CHAPTER OUTLINE
„ Inflammatory Cysts 273
„ Tumors of Odontogenic Origin 275
„ Odontogenic Epithelium with Odontogenic Ectomesenchyme
with or without Hard Tissue Formation 289
Tumors derived from the odontogenic tissues constitute an
unusually diverse group of lesions. This multiformity reflects
the complex development of the dental structures, since
these tumors all originate through some aberration from the
normal pattern of odontogenesis. An understanding of the
pathogenesis of the odontogenic tumors is predicated upon
an understanding of the histogenesis of the tooth.
Certain of the lesions discussed in this chapter represent only
minor alterations in odontogenesis and not true neoplasms.
The odontogenic cysts are included because they too represent
an aberration at some stage of odontogenesis, and in fact, may
be intimately associated with the development of certain of the
odontogenic tumors. All of the various lesions are grouped here
because of their common origin from a uniquely specialized
group of tissues, and their classification is based upon this
origin from the various germ layers.
Odontogenic Cysts
Cysts of the jaws can be classified as:
O Odontogenic (arising from tooth-forming tissues)
O Nonodontogenic (developmental or fissural).
The odontogenic cysts are derived from epithelium
associated with the development of the dental apparatus.
The type of epithelium can vary with most lesions having
stratified squamous but some developmental or fissural cysts
in the maxilla may have respiratory epithelium.
Several types of odontogenic cysts may occur, dependent
chiefly upon the stage of odontogenesis during which they
originate. Various investigators have attempted to devise a
Chapter4.indd 259
„ T"TCLGPFTCP
„ Odontogenic Ectomesenchyme with or without Included
Odontogenic Epithelium 297
„ Malignant Odontogenic Tumors 301
„ Odontogenic Carcinomas 301
classification and system of nomenclature of the lesions. Some
of these classifications have not been entirely satisfactory
because they generally failed to recognize the mode of origin
and development of the cysts and did not unite the views of
the oral surgeon, the radiologist and the pathologist.
The following classification of odontogenic cysts
(Table 4-1) is based on etiology and tissue of origin.
Dentigerous Cyst
(Follicular cyst)
Dentigenous cyst can be defined as an odontogenic cyst that
surrounds the crown of an impacted tooth; caused by fluid
accumulation between the reduced enamel epithelium and
the enamel surface, resulting in a cyst in which the crown is
located within the lumen. This is one of the most common
types of developmental odontogenic cyst, estimated to be about
20% of all jaw cysts. It is estimated that about 10% of impacted
teeth have formed a dentigerous cyst. Their frequency in the
general population has been estimated at 1.44 cyst for every 100
unerupted teeth. The dentigerous cyst nearly always involves
or is associated with the crown of a normal permanent tooth.
Seldom is a deciduous tooth involved.
Clinical Features. This cyst is always associated initially
with the crown of an impacted, embedded or unerupted
tooth (Fig. 4-1). A dentigerous cyst may also be found
enclosing a complex compound odontoma or involving a
supernumerary tooth. The most common sites of this cyst
are the mandibular and maxillary third molar and maxillary
cuspid areas, since these are the most commonly impacted
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Disturbances of Development and Growth

Table 4-1: Classification of odontogenic cysts the absence of these syndromes, bilateral dentigerous cysts are
Classification by etiology
rare. The dentigerous cyst is potentially capable of becoming
an aggressive lesion. Expansion of bone with subsequent
Developmental: Unknown origin but are not the result of an inflammatory reaction
facial asymmetry, extreme displacement of teeth, severe root
Dentigerous cyst resorption of adjacent teeth and pain are all possible sequelae
Eruption cyst
brought about by continued enlargement of the cyst. Cystic
Odontogenic keratocyst
Gingival cyst of newborn involvement of an unerupted mandibular third molar may
Gingival cyst of adult result in a ‘hollowing-out’ of the entire ramus extending up
Lateral periodontal cyst to the coronoid process and condyle as well as in expansion
Calcifying odontogenic cyst of the cortical plate due to the pressure exerted by the lesion.
Glandular odontogenic cyst
Associated with this reaction may be displacement of the
Inflammatory: Result of inflammation third molar to such an extent that it sometimes comes to lie
Periapical cyst compressed against the inferior border of the mandible. In the
Residual cyst case of a cyst associated with a maxillary cuspid, expansion
Paradental cyst of the anterior maxilla often occurs and may superficially
Classification by tissue of origin resemble an acute sinusitis or cellulitis. There is usually no
Derived from rests of Malassez pain or discomfort associated with the cyst unless it becomes
secondarily infected.
Periapical cyst
Residual cyst Radiographic Features. Radiographic examination of the
Derived from reduced enamel epithelium jaw involved by a dentigerous cyst will reveal a radiolucent
area associated in some fashion with an unerupted tooth crown
Dentigerous cyst
Eruption cyst (Fig. 4-2A). The impacted or otherwise unerupted tooth
crown may be surrounded symmetrically by this radiolucency,
Derived from dental lamina (rests of Serres)
although the distinction between a small dentigerous cyst and
Odontogenic keratocyst an enlarged dental follicle or follicular space is quite arbitrary,
Gingival cyst of newborn
Gingival cyst of adult
especially since the small cyst and the enlarged follicle would
Lateral periodontal cyst be histologically identical. While a normal follicular space is
Glandular odontogenic cyst 3–4 mm, a dentigerous cyst can be suspected when the space
Unclassified is more than 5 mm. Only when the size of the radiolucency is
grossly pathologic can the distinction be made with assurance.
Paradental cyst
Calcifying odontogenic cyst Three radiological variations of the dentigerous cyst may
be observed. In the central variety, the crown is enveloped
symmetrically. In these instances, pressure is applied to the
crown of the tooth and may push it away from its direction
of eruption. In this way, mandibular third molars may be
found at the lower border of the mandible or in the ascending
ramus and a maxillary canine may be forced into the maxillary
sinus as far as the floor of the orbit. The lateral type of
dentigerous cyst is a radiographic appearance which results
from dilatation of the follicle on one aspect of the crown.
This type is commonly seen when an impacted mandibular
third molar is partially erupted so that its superior aspect is
exposed. The so-called circumferential dentigerous cyst results
when the follicle expands in a manner in which the entire
tooth appears to be enveloped by cyst. The dentigerous cyst
is usually a smooth, unilocular lesion, but occasionally one
Figure 4-1. Dentigerous cyst. with a multilocular appearance may occur. In actuality, the
The cyst wall is attached to the cervical region of the associated unerupted various compartments are all united by the continuous cystic
tooth. The crown of the tooth projects into the cyst cavity and its roots exhibit
hypercementosis. membrane. Sometimes the radiolucent area is surrounded by
a thin sclerotic line representing bony reaction. In cases of
apparently multiple dentigerous cysts, care should be taken to
rule out the possible occurrence of the odontogenic keratocyst,
teeth. Most lesions present in second and third decades,
basal cell nevus, bifid rib syndrome (q.v.).
SECTION I

with slight male predilection (M:F–3:2). Most dentigerous

cysts are solitary. Bilateral and multiple cysts are usually Histologic Features. There are no characteristic micro-
found in association with a number of syndromes including scopic features which can be used reliably to distinguish the
cleidocranial dysplasia and Maroteaux–Lamy syndrome. In dentigerous cyst from the other types of odontogenic cysts.

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B C
Figure 4-2. Dentigerous cyst.
The radiograph (A) demonstrates a large radiolucent area associated with the crown of the impacted mandibular third molar. The photomicrograph (B) shows this cyst to be
lined by a thin layer of stratified squamous epithelium similar in appearance to the primordial cyst. Occasional mucus-containing cells are present in the epithelium. Small
islands of odontogenic epithelium (C) are often present in the connective tissue wall.
It is usually composed of a thin connective tissue wall with a
thin layer of stratified squamous epithelium lining the lumen
(Fig. 4-2B). Rete peg formation is generally absent except in
cases that are secondarily infected. The connective tissue wall
is frequently quite thickened and composed of a very loose
fibrous connective tissue or of a sparsely collagenized myxo-
matous tissue, each of which has been sometimes mistakenly
diagnosed as either an odontogenic fibroma or an odonto-
genic myxoma. A hyperplastic dental follicle is not necessar-
ily associated with inflammation. An additional feature of the
connective tissue wall of both normal dental follicles and den-
tigerous cysts is the presence of varying numbers of islands
of odontogenic epithelium (Fig. 4-2C). These are sometimes
very sparse and obviously inactive, while at other times they
are present in sufficient numbers to be mistaken for an amelo-
blastoma. While this latter odontogenic tumor can originate
in this situation, care must be taken to differentiate between
it and simply odontogenic epithelial rests. Inflammatory cell
infiltration of the connective tissue is common although the
cause for this is not always apparent. An additional finding,
especially in cysts which exhibit inflammation, is the pres-
ence of Rushton bodies within the lining epithelium. These
are peculiar linear, often curved, hyaline bodies with variable
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Cysts and Tumors of Odontogenic Origin
stainability which are of uncertain origin, questionable nature
and unknown significance. Even electron microscopic studies,
such as those of El-Labban, have only been of partial help in
determining that the structures are probably of hematogenous
origin, although it is not clear why they have such an intimate
relationship to epithelium. The content of the cyst lumen is
usually a thin, watery yellow fluid, occasionally blood tinged.
It was reported by Brannon in his excellent clinicopathologic
study of 312 cases of odontogenic keratocysts that 8.5%
of a series of 1,850 dentigerous cysts were odontogenic
keratocysts (q.v.) with the characteristic histologic findings
in the epithelium of a parakeratinized, corrugated surface, a
remarkable uniformity of a 6- to 10-cell thickness without rete
peg formation, and a polarized and palisaded basal layer of cells.
This percentage of dentigerous cysts which are odontogenic
keratocysts is in close agreement with the findings of Pindborg
and his coworkers (7.1%) and Payne (8.5%).
The pluripotentialities of the epithelium in mandibular
dentigerous cysts has been further emphasized by Gorlin,
SECTION I
who described mucus-secreting cells in the lining stratified
squamous epithelium, respiratory epithelial lining, sebaceous
cells in the connective tissue wall, and lymphoid follicles with
germinal centers.
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Disturbances of Development and Growth

Treatment. The treatment of the dentigerous cyst is usually

dictated by the size of the lesion. Smaller lesions can be
surgically removed in their entirety with little difficulty. The
larger cysts which involve serious loss of bone and thin the
bone dangerously are often treated by insertion of a surgical
drain or marsupialization. Such a procedure is often necessary
because of the potential danger of fracturing the jaw if
complete surgical removal were attempted. Recurrence is
relatively uncommon.
Potential Complications. Several relatively serious poten-
tial complications exist stemming from the dentigerous cyst,
besides simply the possibility of recurrence following incom-
plete surgical removal. These include:
O The development of an ameloblastoma either from the lin-
A
ing epithelium or from rests of odontogenic epithelium in
the wall of the cyst.
O The development of epidermoid carcinoma from the same
two sources of epithelium.
O The development of a mucoepidermoid carcinoma, ba-
sically a malignant salivary gland tumor, from the lining
epithelium of the dentigerous cyst which contains mucus-
secreting cells, or at least cells with this potential, most
commonly seen in dentigerous cysts associated with im-
pacted mandibular third molars.

It is of great clinical significance that numerous cases of

ameloblastoma have been reported developing in the wall of
dentigerous cysts from the lining epithelium or associated
epithelial rests (Fig. 4-3). Stanley and Diehl have reviewed
a series of 641 cases of ameloblastoma and have found that
at least 108 cases of this neoplasm, approximately 17%,
were definitely associated with an impacted tooth and/or a
follicular or dentigerous cyst. The disposition for neoplastic
epithelial proliferation in the form of an ameloblastoma is far
more pronounced in the dentigerous cyst than in the other
odontogenic cysts. The formation of such a tumor manifests
itself as a nodular thickening in the cyst wall, the mural
ameloblastoma, but this is seldom obvious clinically. Therefore,
it is not only good clinical practice, but also an absolute
requisite that all tissue from dentigerous cysts be submitted to
a qualified oral pathologist for thorough gross and microscopic
examination. In reviewing the histologic changes sought by
the oral pathologist which occur in the dentigerous cyst as
a premonitory manifestation of ameloblastoma, Vickers and
Gorlin have stressed that hyperchromatism of basal cell nuclei,
palisading with polarization of basal cells and cytoplasmic
vacuolization with intercellular spacing of the lining epithelium,
when observed together, are manifestations of impending
neoplasia. These findings may occur individually in other
rather harmless conditions. The presence of sprouting or
budding and protruding of epithelial islands from lining
epithelium has been claimed to be evidence of neoplastic
SECTION I
B
Figure 4-3. Ameloblastoma developing in wall of dentigerous cyst.
documented in the literature reviewed by Gardner, who
reported eight acceptable cases among 25 cases of carcinoma
developing in odontogenic cysts of all types combined. Browne
and Gough have reported two additional cases of malignant
transformation in dental cysts and suggested that keratin
metaplasia in long-standing cyst lining appears to precede the
development of the carcinomatous change, although there is
little evidence that the odontogenic keratocyst is associated
with malignant change more commonly than other types of
odontogenic cysts. The predisposing factor and mechanism
of development of this malignancy are unknown, but its
occurrence appears unequivocal (Fig. 4-4).
Finally, the development of a mucoepidermoid carcinoma,
a type of salivary gland tumor, is less well documented than
the epidermoid carcinoma of this origin, but it also appears
to be a potentiality. The inclusion of normal salivary gland
tissue in the posterior portion of the body of the mandible
has been reported, and undoubtedly, some central salivary
gland tumors in this location originate from this source.

transformation, but this in itself was not considered such an However, cases of central mucoepidermoid carcinoma (q.v.)
indication by Vickers and Gorlin. have been discovered in association with dentigerous cysts
The development of epidermoid carcinoma from the lining involving impacted mandibular third molars, and considering
epithelium of the dentigerous cyst also has been adequately the frequency with which mucus-secreting cells are found

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A B
Figure 4-4. Epidermoid carcinoma developing in dentigerous cyst.
in this lining epithelium indicative of the pluripotentiality
of this epithelium, this very distinct possibility must always
be considered. These findings comprise most of the medical
rationale for removal of impacted third molars with pericoronal
radiolucencies; however, impacted teeth with small pericoronal
radiolucencies (suggesting the presence of normal dental follicle
rather than dentigerous cyst) also may be monitored with serial
radiographic examination. Any increase in the size of the lesion
should prompt removal and histopathologic examination. Any
lesion that appears larger than a normal dental follicle indicates
removal and histopathologic examination.
Eruption Cyst
Eruption cyst is defined as an odontogenic cyst with the
histologic features of a dentigerous cyst that surrounds a tooth
crown that has erupted through bone but not soft tissue and
is clinically visible as a soft fluctuant mass on the alveolar
ridges.
An eruption cyst or ‘eruption hematoma’ is in fact a
dentigerous cyst occurring in the soft tissues (Shear, 1992).
Whereas the dentigerous cyst develops around the crown
of an unerupted tooth lying in the bone, the eruption cyst
occurs when a tooth is impeded in its eruption within the soft
tissue overlying the bone. The pathogenesis is probably very
similar to that of the dentigerous cyst. The difference is that
the tooth in the case of the eruption cyst is impeded in the
soft tissue of gingiva rather than in the bone. The presence
of particularly dense fibrous tissue in the overlying gingiva
could be responsible.
Clinical Features. Shear (1992) had recorded a 0.8%
frequency. It is likely that they occur more frequent clinically
and as some rupture spontaneously, these cysts go unnoticed
and are not submitted for histological examination.
These cysts are found in children of different ages, and
occasionally in adults if there is delayed eruption. Deciduous
and permanent teeth may be involved, most frequently anterior
to the first permanent molar. Clinically, the lesion appears as a
circumscribed, fluctuant, often translucent swelling of the
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263
Cysts and Tumors of Odontogenic Origin
alveolar ridge over the site of the erupting tooth. When the
circumcoronal cystic cavity contains blood, the swelling appears
purple or deep blue; hence the term ‘eruption hematoma’.
It is usually painless unless infected.
Sometimes more than one cyst may be present. There is
often a brief history of about three to four weeks duration
during which they enlarge to approximately 1–1.5 cm.
Radiographic Features. It may show a soft tissue shadow
since the cyst is confined within it and there is usually no bone
involvement.
Histologic Features. The superficial aspect is covered by
the keratinized stratified squamous epithelium of the overly-
ing gingiva. This is separated from the cyst by a strip of dense
connective tissue of varying thickness which usually shows a
mild chronic inflammatory cell infiltrate. Inflammatory cell
infiltration is common. The follicular connective tissue is
more densely cellular, less collagenous and has a basophilic
hue, presumably because of a higher content of acid muco-
polysaccharide in the ground substance. Odontogenic epithe-
lial cell nests may be present in the connective tissue.
In noninflamed areas, the epithelial lining of the cysts is
characteristically of reduced enamel epithelial origin, consisting of
two or three cell layers of squamous epithelium with a few foci
where it may be a little thicker. The adjacent corium is hyperemic
and is the seat of a chronic inflammatory cell infiltrate (Fig. 4-5).
Treatment. No treatment is necessary as the cyst often
ruptures spontaneously. Surgically exposing the crown of the
tooth may aid the eruption process.
Odontogenic Keratocyst
A cyst derived from the remnants (rests) of the dental lamina,
with a biologic behavior similar to a benign neoplasm, with
a distinctive lining of six to 10 cells in thickness, and that
SECTION I
exhibits a basal cell layer of palisaded cells and a surface of
corrugated parakeratin.
This is the most interesting of jaw cysts. The term ‘odontogenic
keratocyst’ was first used by Philipsen in 1956, while Pindborg and
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Disturbances of Development and Growth
A B
Figure 4-5. Eruption cyst.
The cyst is lined by a thin layer of stratified squamous epithelium in which there are scattered inflammatory cells. Eruption cyst often shows marked secondary inflammatory
changes from masticatory trauma.
Hansen in 1963 described the essential features of this type of cyst.
It is named keratocyst because the cyst epithelium produces so
much keratin that it fills the cyst lumen. Furthermore, flattening
of the basement membrane and palisading of the basal epithelial
cells, reminiscent of odontogenic epithelium, are characteristics
of odontogenic keratocyst.
They are unique odontogenic lesions that have the potential
to behave aggressively, that can recur, and can be associated
with the nevoid basal cell carcinoma syndrome. Toller (1967)
suggested that OKCs might be regarded as benign cystic
neoplasms. Whether they are developmental or neoplastic
continues to be debated.
Studies indicate that a significant number of OKCs show
clonal loss of heterozygosity of common tumor suppressor
genes. The finding of clonal deletion mutations of genomic
DNA in these cysts supports the hypothesis that they are
neoplastic rather than developmental in origin. The odonto-
genic keratocyst is regarded as a distinctive entity because of
its characteristic histology, proliferation kinetics, and behavior.
Therefore, although keratinization may be present in many
other types of cysts, the specific histologic pattern of the
odontogenic keratocyst separates it from all others.
Differences in cytokeratin, epithelial membrane antigen
(EMA) and carcinoembryonic antigen (CEA) immunoreactiv-
ity between the parakeratinized OKC and the orthokeratinized
variety have been demonstrated and the suggestion made that
the latter having a considerably less aggressive behavior is
different entity and should bear a different name orthoke-
ratinized odontogenic cyst (Shear M, 2002).
There is general agreement that the origin of the odontogenic
SECTION I
keratocyst comes from dental lamina remnants in the mandible
and maxilla. However, the origin of this cyst from the extension
of basal cells of the overlying oral epithelium has also been
suggested.
Chapter4.indd 264
Reclassification of the Odontogenic Keratocyst to
Tumor: Keratocystic Odontogenic Tumor (KOT)
In 1967, Toller suggested that the OKC may best be regarded
as a benign neoplasm rather than a conventional cyst based
on its clinical behavior. The WHO has reclassified the lesion
as a tumor based on several factors, that formed the basis of
this decision.
O Behavior: The KOT is locally destructive and recurrence
rate is very high.
O Histopathology: The basal epithelial layer of KOT shows
proliferation and budding into the underlying connective
tissue in the form of daughter cysts and mitotic figures are
frequently found in the suprabasal layers of the lesional
epithelium.
O Genetics: PTCH (patched), a tumor suppressor gene in-
volved in both syndrome associated and sporadic KOTs,
occurs on chromosome 9q22.3 – q31. Normally, PTCH
forms a receptor complex with the oncogene SMO
(smoothened) for the SHH (sonic hedgehog) ligand.
PTCH binding to SMO inhibits growth signal transduc-
tion. SHH binding to PTCH releases this inhibition. If
normal functioning of PTCH is lost, the proliferation-
stimulation effects of SMO are permitted to predominate.
Evidence has shown that the pathogenesis of syndrome
associated and sporadic KOTs involves a ‘two hit mechanism’,
with allelic loss at 9q22. The ‘two hit mechanism’ refers to
the process by which a tumor suppressor gene is inactivated.
The first hit is a mutation in one allele, which, although it
can be dominantly inherited, has no phenotypic effect. The
second hit refers to loss of the other allele and is known as
‘loss of heterozygosity’ (LOH). In KOTs, this leads to the
dysregulation of the oncoproteins cyclin D1 and p53. LOH
01/06/12 10:54 AM

in the 9q22.3–q31 region has been reported for many epithe-
lial tumors, including basal cell carcinomas, squamous cell
carcinomas, and transitional cell carcinomas; and LOH is by
definition a feature of tumorigenic tissue.
Clinical Features. The largest and most detailed series
of cases of odontogenic keratocyst has been published by
Brannon, and his data are probably most representative of this
lesion. The cyst may occur at any age, from the very young to
the very elderly, although Brannon found it to be exceedingly
rare under the age of 10 years, there being only two such
patients in his series of 283 persons. The peak incidence is
in the second and third decades of life, with a gradual decline
thereafter. The data of Browne (104 patients) and of Forssell
(119 patients) are virtually identical. In all series, there is a
predilection for occurrence in males, ranging from 1.44:1
(Brannon), 1.46:1 (Browne) to 1.79:1 (Forssell).
The mandible is invariably affected more frequently than
the maxilla: in the series of Brannon, 65% versus 35%, in the
series of Browne, 79% versus 21%, and in that of Forssell,
78% versus 22%. In the mandible, the majority of the cysts
occur in the ramus-third molar area, followed by the first
and second molar area and then the anterior mandible. In
the maxilla, the most common site is the third molar area
followed by the cuspid region.
Multiple odontogenic keratocysts occur with some
frequency. Lesions found in children are often reflective of
multiple odontogenic keratocysts as a component of the nevoid
basal cell carcinoma syndrome. However, at other times, these
multiple cysts are independent of the syndrome. A rather
remarkable parallelism between the odontogenic keratocyst and
the ameloblastoma with respect to mean age of occurrence,
predilection for site of occurrence, radiographic findings and
recurrence rate has also been pointed out by Browne.
There are no characteristic clinical manifestations of the
keratocyst, although about 50% of the patients in Brannon’s series
were symptomatic prior to seeking treatment. Among the more
common features are pain, soft-tissue swelling and expansion
of bone, drainage and various neurologic manifestations such as
paresthesia of the lip or teeth. The maxillary OKC tends to be
secondarily infected with greater frequency than the mandibular
ones, due to its vicinity to the maxillary sinus.
Radiographic Features. Radiographically most OKCs are
unilocular, presenting a well-defined peripheral rim. Scalloping
of the border is also a frequent finding and this represents varia-
tions in the growth pattern of the cyst. Multilocular radiolucent
OKC is also observed, generally representing a central cavity
having satellite cysts. When it is multilocular and especially if
located in the third mandibular molar area, it may be confused
radiographically with an ameloblastoma. Occasionally OKC
may mimic a dentigerous cyst and contain the crown of a re-
tained tooth within its lumen. The final diagnosis of any cystic
cavity within the jaw bones will be achieved only after biopsy
of the surgical specimen. Multilocularity (20%) is often present
and tends to be seen more frequently in larger lesions. Most
lesions, however, are unilocular, with as many as 40% noted
adjacent to the crown of an unerupted tooth (dentigerous cyst
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265
Cysts and Tumors of Odontogenic Origin
position). Approximately 30% of maxillary and 50% of man-
dibular lesions produce buccal expansion. Mandibular lingual
enlargement is occasionally seen. Proximity to the roots of ad-
jacent normal teeth sometimes causes resorption of these roots,
although displacement is more common. Sometimes these
cysts displace the neurovascular bundle.
Histologic Features. The odontogenic keratocyst wall is
usually rather thin unless there has been superimposed in-
flammation. The lining epithelium is highly characteristic,
and is composed of:
O A parakeratinized surface which is typically corrugated,
rippled or wrinkled.
O A remarkable uniformity of thickness of the epithelium,
usually ranging from 6 to 10 cells thick.
O A prominent palisaded, polarized basal layer of cells often de-
scribed as having a ‘picket fence’ or ‘tombstone’ appearance.
Histologically, these cysts are formed with a stratified
squamous epithelium that produces orthokeratin (10%),
parakeratin (83%), or both types of keratin (7%). No rete ridges
are present; therefore, the epithelium often sloughs from the
connective tissue (94% of the time). The epithelium is thin,
and mitotic activity is frequent; therefore, OKC grows in a
neoplastic fashion and not in response to internal pressure. In
the presence of an intense inflammatory process, the adjacent
epithelium loses its keratinized surface, may thicken and
develop rete processes or may ulcerate.
The connective tissue wall often shows small islands of
epithelium similar to the lining epithelium; some of these
islands may be small cysts. In at least some cases, the apparent
islands of epithelium and small satellite or ‘daughter’ cysts
actually represent the ends of folds of the lining epithelium
of the main cystic cavity which have been cut in cross-section;
the linings of these cysts are very commonly folded. Forssell
and his associates have studied this problem using serial
sections of cysts and found microcysts in the wall in 20% and
epithelial islands in 50% of their cases. In 35% of the cases,
apparent microcysts were found to be part of the main cyst
by the serial sections while pseudo islands of epithelium were
found in 75% of the cases.
The lumen of the keratocyst may be filled with a thin straw
colored fluid or with a thicker creamy material. Sometimes the
lumen contains a great deal of keratin, while at other times
it has little. Cholesterol, as well as hyaline bodies at the sites
of inflammation, may also be present. The electrophoretic
measurement of fluid from these cysts has been reported by
Toller to show that it contains a very low content of soluble
protein compared with the patient’s own serum (Fig. 4-6).
Dysplastic and neoplastic transformation of the lining
epithelium in the odontogenic keratocyst is an uncommon
occurrence but has been reported. Of the 312 keratocysts studied
by Brannon, only two exhibited cellular atypia. Occasional
other cases have also been described in the literature. Areen
SECTION I
and his associates have recently reported a case of epidermoid
carcinoma developing in an odontogenic keratocyst, and in
reviewing the literature, have emphasized the necessity for
careful microscopic examination of all such cysts.
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Disturbances of Development and Growth
A
B
Figure 4-6. Odontogenic keratocyst.
The variant of OKC that produces only orthokeratin acts
somewhat differently than other OKCs. These almost always
are found in a dentigerous association, usually around the
mandibular third molar, and they are much less aggressive.
They do not have a hyperchromatic basal layer; in fact, the
basal layer is flattened. They are not associated with basal cell
nevus syndrome (orthokeratinized odontogenic cyst).
Finally, the highly characteristic nature of the parakeratinized
lining epithelium and its relationship to the high recurrence rate
have been emphasized by a report dealing with orthokeratinized
odontogenic cysts and their recurrence rate. Wright investigated
59 cases of orthokeratinized odontogenic cysts and found that
they showed a predilection for occurrence in males, most
commonly in the second to fifth decades. These cysts were
located predominantly in the posterior mandible, where they
most typically appeared as dentigerous cysts. The thin, uniform
lining epithelium was covered with orthokeratin and showed
a prominent granular layer and a cuboidal to flattened basal
layer. Follow-up of 24 of these patients revealed only one
case of recurrence. This difference in biologic behavior would
further underscore the necessity for very strict application of
the definition of the term odontogenic keratocyst in diagnosis
of the lesion (Fig. 4-7).
SECTION I
Treatment and Prognosis. The odontogenic keratocyst
should be surgically excised. However, clinical experience has
shown that complete eradication of the cyst may be difficult
because the wall of the cyst is very thin and friable and may
Chapter4.indd 266
A
B
Figure 4-7. Odontogenic keratocyst.
(A) The epithelial lining is uniformly thin, generally ranging from 8–10 cell layers thick.
The basal layer exhibits a characteristic palisaded pattern with polarized and intensely
stained nuclei of uniform diameter. The luminal epithelial cells are parakeratinized
and produce an uneven or corrugated profile. (B) Odontogenic keratocyst stained
using proliferating cell nuclear antigen (PCNA) antibodies (x 10).
easily fragment. In addition, perforation of cortical bone,
particularly in lesions involving the ramus, is common and
this complicates total removal.
The most important feature of the odontogenic keratocyst
is its extraordinary recurrence rate. This has been reported
as being between 13 and 60%. A review of 763 cases of
odontogenic keratocysts in 13 different reported series of
cases has shown the average recurrence rate to be 26%,
with the majority occurring within five years of the surgical
procedure. Forssell, Forssell and Kahnberg (1988) observed
that recurrences were more frequent (63%) with cysts in
patients with the nevoid basal cell carcinoma syndrome than
with cysts in patients without the syndrome (37%). Keratocysts
enucleated in one piece recurred significantly less often than
cysts enucleated in several pieces, and the recurrence rate in
cases with a clinically observable infection, a fistula or with
a perforated bony wall was higher. The size of the cyst did
not seem to influence its prognosis after surgery, but those
whose radiographic appearance was multilocular had a higher
recurrence rate than those with a unilocular appearance.
Browne found no significant differences in recurrence rate
following three basic methods of treating the lesions:
O Marsupialization
01/06/12 10:54 AM

O Enucleation and primary closure
O Enucleation and packing open.
Furthermore, recurrence does not appear related to the
presence of satellite cysts. On this basis, Browne concluded
that recurrence of the keratocyst is due to the nature of the
lesion itself, i.e. the presence of additional remnants of dental
lamina from which a cyst may develop, and is not related to
its method of treatment. Since recurrence may be long delayed
in this lesion, follow-up of any case of odontogenic keratocyst
with annual radiographs is essential for at least five years after
surgery. It is also essential that patients with an odontogenic
keratocyst, especially if multiple, be evaluated medically to
rule out the possibility of the jaw cyst-basal cell nevus-bifid
rib syndrome (q.v.).
Jaw Cyst-Basal Cell Nevus, Bifid Rib Syndrome
(Basal cell nevus syndrome, hereditary cutaneomandibular
polyoncosis, Gorlin and Goltz syndrome)
This syndrome, first described by Binkley and Johnson in 1951,
has been thoroughly reviewed by Gorlin and his coworkers. A
hereditary condition, it is transmitted as an autosomal dominant
trait, with high penetrance and variable expressivity. It is caused
by mutations in patched (PTCH), a tumor suppressor gene that
has been mapped to chromosome 9q22.3–q31.
Clinical Features. The syndrome is very complex and
includes a great variety of possible abnormalities. These may
be briefly summarized as follows:
O Cutaneous anomalies, including basal cell carcinoma,
other benign dermal cysts and tumors, palmar pitting, pal-
mar and plantar keratosis and dermal calcinosis.
O Dental and osseous anomalies, including odontogenic
keratocysts (often multiple), mild mandibular progna-
thism, rib anomalies (often bifid), vertebral anomalies and
brachymetacarpalism (Fig. 4-8).
O Ophthalmologic abnormalities, including hyper-
telorism with wide nasal bridge, dystopia canthorum, con-
genital blindness and internal strabismus.
O Neurologic anomalies, including mental retardation,
dural calcification, agenesis of corpus callosum, congenital
hydrocephalus and occurrence of medulloblastomas with
greater than normal frequency.
O Sexual abnormalities, including hypogonadism in males
and ovarian tumors.
Some of these patients have shown a lack of response to
parathormone as judged by the lack of phosphate diuresis
which, coupled with shortened fourth metacarpals in some
patients, has suggested that there may be some relationship
to pseudohypoparathyroidism.
Oral Manifestations. The odontogenic keratocysts are
indistinguishable from those previously described by that term
which are not associated with this syndrome (Fig. 4-8). Because
they often develop early in life, deformity and displacement of
developing teeth may occur. However, they may not develop
until middle age even though the basal cell skin tumors have
occurred early in life.
Chapter4.indd 267
267
Cysts and Tumors of Odontogenic Origin
Figure 4-8. Odontogenic keratocysts in the basal cell nevus syndrome.
Treatment and Prognosis. Several cases of ameloblastoma
have developed in cysts of this syndrome, thus emphasizing
the importance of surgical removal of the cysts and their
histologic examination. Whenever a diagnosis of odontogenic
keratocyst is received by the dentist, he must be certain to
rule out the presence of this syndrome because of the many
associated problems which these patients ultimately will face.
Dental Lamina Cyst of Newborn
(Gingival cyst of newborn, Epstein’s pearls, Bohn’s nodules)
Dental lamina cyst of the newborn are multiple, occasionally
solitary, superficial raised nodules on edentulous alveolar
ridges of infants that resolve without treatment; derived from
rests of the dental lamina and consisting of keratin-producing
epithelial lining. Bohn’s nodules and Epstein’s pearls are
two similar lesions with which gingival cysts sometimes
may be confused; however, the location and etiology of
these lesions are somewhat different. As originally described,
Epstein’s pearls are cystic, keratin-filled nodules found along
the midpalatine raphe, probably derived from entrapped
epithelial remnants along the line of fusion (q.v.). Bohn’s
nodules are keratin-filled cysts scattered over the palate, most
numerous along the junction of the hard and soft palate and
apparently derived from palatal salivary gland structures (q.v.).
Discussions of these various types of cysts in the newborn
have been published by Fromm and by Cataldo and Berkman.
In studying sections of the maxillae and mandibles of 17
infants, Kreshover reported finding 65 examples of gingival
cysts (38 multiple and 27 single). These cysts were localized in
the corium below the surface epithelium. Those in the anterior
portion of the jaws were usually displaced lingually with respect
to the deciduous incisors and cuspids. Those in the posterior
portion of the jaw were found occlusal to the crown of the
molars. Kreshover stated that in all instances the cystic lesions
were seen to arise from cells of the dental lamina. The etiology of
these cysts has been thoroughly discussed by Maher and Swindle.
Clinical Features. Occasionally these dental lamina cysts
in infants become sufficiently large to be clinically obvious as
small discrete white swellings of the alveolar ridge, sometimes
appearing blanched as though from internal pressure
SECTION I
(Fig. 4-9A). These probably correspond to those structures
described in the older literature as the ‘predeciduous
dentition’. These lesions appear to be asymptomatic and do
not seem to produce discomfort in the infants.
01/06/12 10:54 AM
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Disturbances of Development and Growth
A B
Figure 4-9. Gingival cyst of the newborn.
(Courtesy of Dr Ralph E McDonald and Dr Alfred Fromm).
Histologic Features. These are true cysts with a thin epi-
thelial lining which lacks rete processes and show a lumen
usually filled with desquamated keratin, occasionally contain-
ing inflammatory cells (Fig. 4-9B). Interestingly, dystrophic
calcification and hyaline bodies of Rushton (q.v.), commonly
found in dentigerous cysts, are also sometimes found in this
lesion.
Treatment. No treatment is required inasmuch as these
lesions almost invariably will disappear by opening onto the
surface of the mucosa or through disruption by erupting teeth.
Gingival Cyst of Adult
A small developmental odontogenic cyst of the gingival soft
tissue derived from the rests of the dental lamina, containing a
lining of embryonic epithelium of cuboidal cells and distinctive
focal thickenings similar to the lateral periodontal cyst.
The gingival cyst of the adult is an uncommon cyst of gingival
soft tissue, occurring in either the free or attached gingiva.
The etiology and pathogenesis of this lesion have been
reviewed by Ritchey and Orban, who suggested possible
sources of cystic formation as:
O Heterotopic glandular tissue.
O Degenerative changes in a proliferating epithelial peg.
O Remnants of the dental lamina, enamel organ or epithelial
islands of the periodontal membrane.
O Traumatic implantation of epithelium.
Of these possibilities, only the last two appear valid, and
on this basis there do appear to be two recognizable forms
of gingival cyst:
O That arising from cystic transformation of dental lamina or
the ‘glands’ or rests of Serres.
O That arising from traumatic implantation of surface epithe-
lium (and, therefore, not truly an odontogenic cyst).
The origin of the gingival cyst of the adult has been
SECTION I
evaluated by Wysocki and his colleagues, who concluded that
it does arise from postfunctional rests of dental lamina and
thus represents the extraosseous counterpart of the lateral
periodontal cyst, with which it shares a common histogenesis.
Chapter4.indd 268
Buchner and Hansen have reported essentially the same
conclusions. The similarities between the lateral periodontal
cyst and the gingival cyst of the adult in clinical behavior,
morphologic appearance, anatomic site of occurrence and age
predilection are too striking to be coincidental.
The vast majority of these gingival cysts appear to originate
in the fashion described from dental lamina, including a soft
tissue counterpart of the multilocular botryoid odontogenic
cyst. However, an implantation type of cyst can occur lined
by a more mature keratinizing stratified squamous epithelium
lining derived from surface mucosal epithelium. Finally, as
suggested by Buchner and Hansen, there is some evidence
that a dental lamina cyst of the newborn may persist into
adulthood, as judged by the finding of cysts packed with
orthokeratin that appear identical to those in the newborn.
Clinical Features. The gingival cyst may occur at any age
but is most common in adults. In the review of the literature
by Reeve and Levy, the majority of patients were over 40 years
of age. The mean age in the 33 cases reported by Buchner
and Hansen was 48 years and that of the 10 cases reported
by Wysocki and his associates was 51 years. The location of
the lesion closely follows that of the lateral periodontal cyst.
Thus, all except one cyst in the series of Wysocki and his
coworkers were in the mandibular bicuspid-cuspid incisor
area, the one exception being in the maxillary lateral incisor
area. The locations in the series of Buchner and Hansen were
virtually identical except that they had several cases also in the
maxillary arch from cuspid to first molar.
This lesion presents generally as a small, well-circumscribed,
painless swelling of the gingiva, sometimes closely resembling
a superficial mucocele (Fig. 4-10). The lesion is of the same
color as the adjacent normal mucosa and seldom measures
over 1 cm in diameter, generally much less. Although this
cyst may occur in either the free or attached gingiva, some
gingival cysts occur in the gingival papilla itself.
Radiographic Features. The gingival cyst is a soft tissue
lesion and does not generally manifest itself on the dental
radiograph. If it enlarges to sufficient size, it may cause
superficial erosion of the cortical plate of bone, but this is still
generally not visible on the radiograph. If a circumscribed,
01/06/12 10:54 AM

A area. The possibilities which have been offered to explain their
B tion is unknown.
Figure 4-10. Gingival cyst of the adult.
radiolucent cystic lesion of alveolar bone with some swelling
of the soft tissue is present, the cyst probably represents a
lateral periodontal cyst rather than a gingival cyst.
Histologic Features. The gingival cyst of the adult is a true
cyst, since it is a pathologic epithelium lined cavity which
usually contains fluid (Fig. 4-10 B). The lining epithelium is
generally identical to that found in the lateral periodontal cyst,
with the occasional exceptions noted above. The epithelium
ranges in thickness from simply one flattened cell to several
cells, a thin stratified squamous epithelium. Glycogen-rich
clear cells may be present, especially in the focal thickenings or
plaques of the lining. Dental lamina rests may also be found in
the connective tissue wall and these are commonly composed
of the same type of glycogen-rich clear cells. As noted earlier,
these lesions may be unicystic or polycystic. Since the cyst lies
free in the connective tissue of the gingiva, it may or may not
exhibit an associated inflammatory reaction.
In cases of the traumatic or implantation type of gingival
cyst, calcification or even ectopic ossification on rare occasions
may be associated with the cystic lesion, reminiscent of
the ossification occurring after experimental implantation of
bladder epithelium into subcutaneous tissues.
Treatment. Local surgical excision of the lesion in adults is
usually recommended, and the lesions do not tend to recur. A
neoplastic potential has never been reported.
Lateral Periodontal Cyst
A slow growing, nonexpansile developmental odontogenic
cyst derived from one or more rests of the dental lamina,
containing an embryonic lining of one to three cuboidal cells
and distinctive focal thickenings (plaques).
Chapter4.indd 269
269
Cysts and Tumors of Odontogenic Origin
The lateral periodontal cyst, as the name implies, occurs on
a lateral periodontal location and it is of developmental origin
arising from cystic degeneration of clear cells of the dental lamina.
In order to establish the proper diagnosis, an inflammatory origin
as well as exclusion of a possible odontogenic keratocyst must
be ruled out clinically and histologically.
The lateral periodontal cyst is an uncommon but well-
recognized type of developmental odontogenic cyst. The various
theories concerning the etiology and pathogenesis of the lesion
have been reviewed by Standish and Shafer. These cysts appear
to arise in intimate association with the lateral root surface of an
erupted tooth, with a predilection for the mandibular bicuspid
origin and mode of development include:
O Origin initially as a dentigerous cyst developing along the
lateral surface of the crown, and as the tooth erupts, the
cyst assumes a position in approximation to the lateral sur-
face of the root.
O Origin from proliferation of rests of Malassez in the peri-
odontal ligament although the stimulus for this prolifera-
O Origin simply as a primordial cyst of a supernumerary
tooth germ, since the predilection for occurrence of the
lateral periodontal cyst in the mandibular bicuspid area
corresponds well with the known high incidence of super-
numerary teeth in this same region.
O Origin from proliferation and cystic transformation of rests
of dental lamina, which are in a postfunctional state and
therefore have only a limited growth potential that is in ac-
cordance with the usual small size of these cysts.
This latter theory, including the suggestion that the lateral
periodontal cyst and the gingival cyst of the adult (q.v.) share
this common histogenesis from postfunctional dental lamina
rests and that these two cysts represent basically the central
or intraosseous and peripheral or extraosseous manifestations
of the same lesion, has been discussed in detail by Wysocki
and his colleagues. At present, it seems the most appropriate
one. They have also pointed out the important fact that in
many reports of the lateral periodontal cyst in the previous
literature, the term has been used to designate any cyst that
may be positioned against the lateral root surface of a tooth
(e.g. lateral radicular cyst related to pulp infection, odontogenic
keratocyst, etc.). This positional use of the term should be
avoided and the designation applied only to that specific
developmental lesion with characteristic features.
An unusual form of cyst was reported in 1973 by Weathers
and Waldron under the term botryoid odontogenic cyst.
They described two cases of cysts which had a multilocular
pattern apparent radiographically, histologically and even
clinically at the time of surgical removal. Additional experience
with this cyst, as indicated by Wysocki and his associates,
now strongly suggests that this is simply a polycystic variant
SECTION I
of the lateral periodontal cyst developing through cystic
transformation of multiple islands of dental lamina rests. The
epithelial lining in the two cysts is identical, as are its clinical
features, including age of predilection and sites of occurrence.
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Disturbances of Development and Growth
In addition, a multilocular extraosseous form analogous to the
gingival cyst of the adult also occurs.
Clinical Features. The lateral periodontal cyst occurs
chiefly in adults, according to the series of 39 cases reported
by Wysocki and his associates in which there was a mean age
of 50 years and an age range of 22–85 years. In this series, there
was a predilection for occurrence in males over females, 67: 28
with 5% unknown. The location of the lesion was extremely
limited in this study: 67% of cases were in the mandibular
bicuspid/cuspid/incisor area, while 33% were in the maxillary
lateral incisor area. Lesions were found at no other sites and
this has also been the experience of most other investigators.
No rational explanation has been offered for this localization.
The majority of cases have presented no clinical signs
or symptoms and have been discovered during routine
radiographic examination of the teeth. Occasionally, when the
cyst is located on the labial surface of the root, there may be a
slight mass obvious, although the overlying mucosa is normal.
Unless otherwise affected, the associated tooth is vital. If the
cyst becomes infected, it may resemble a lateral periodontal
abscess and even seek to establish drainage.
Radiographic Features. The periapical radiograph discloses
the lateral periodontal cyst as a radiolucent area in apposition
to the lateral surface of a tooth root (Fig. 4-11A–D). The
lesion is usually small, seldom over 1 cm in diameter, and may
or may not be well circumscribed. In most cases the border is
A B
SECTION I
C D
Figure 4-11. Lateral periodontal cysts.
A cyst lies in the periodontal tissues proximal to the roots of premolar and molar teeth. The pulp of the associated teeth appear normal.
Chapter4.indd 270
definitive and is even surrounded sometimes by a thin layer of
sclerotic bone. The botryoid odontogenic cyst appears similar
except that its polycystic nature is often evident through its
multilocular pattern on the radiograph (Fig. 4-12).
Histologic Features. Histologically, the lateral periodontal
cyst is a distinct type of developmental cyst characterized by
a thin, nonkeratinized epithelium usually one to five cell lay-
ers thick, which resembles the reduced enamel epithelium.
Cuboidal or even columnar cells may be found composing
the lining. Many of the lining cells have a clear, vacuolated,
glycogen-rich cytoplasm (Fig. 4-13). This lining is incomplete
and easily sloughs away. Focal thickened plaques of proliferat-
ing lining cells often project into the lumen in areas. These
are especially prominent in the botryoid odontogenic cyst
(Fig. 4-13). Rests of dental lamina are sometimes found in
the connective tissue wall and these similarly are frequently
composed of glycogen-rich clear cells. These also appear to be
more common in the botryoid-type cyst. Papillary infoldings
of the lateral periodontal cyst wall are sometimes seen and in-
flammatory cells may be present, but this is a secondary reac-
tion. The histologic characteristics of these cysts have been
detailed by Wysocki and his colleagues as well as by Shear and
Pindborg. The connective tissue subjacent to the epithelium
exhibits a zone of hyalinization, consisting of a thick fibrous
noninflamed cyst wall. A different histologic appearance
has been described for this cyst with the name of botryoid
01/06/12 10:54 AM
 271
Cysts and Tumors of Odontogenic Origin

A B

C D

Figure 4-12. Botryoid odontogenic cysts.

The multilocular appearance (A, B) is indicative of their polycystic nature. The lining epithelium (C, D) of the numerous small cysts is thin and shows focal areas of
thickening.

SECTION I

A B
Figure 4-13. Lateral periodontal cysts.

Chapter4.indd 271 01/06/12 10:54 AM

 272
Disturbances of Development and Growth
odontogenic cyst. This name reflects the gross similarity of
the cystic cavities to that of a cluster of grapes.
Treatment and Prognosis. Provided that the lesion is
unilocular on radiographic examination the lateral periodontal
cyst is treated by surgical enucleation. Attempts should be
made to avoid sacrificing the associated tooth, but this may
not always be possible. The botryoid variety, a lesion that is
radiographically or histologically multilocular, has an increased
risk of recurrence or persistence and patients treated for a
botryoid odontogenic cyst should be followed periodically.
It is especially important that the diagnosis be established
because of the similarity in appearance between this cyst and
other more serious lesions such as an early ameloblastoma.
Calcifying Odontogenic Cyst
(Keratinizing and/or calcifying epithelial odontogenic cyst, Gorlin
cyst, cystic keratinizing tumor)
A rare, well-circumscribed, solid or cystic lesion derived from
odontogenic epithelium that resembles follicular ameloblastoma
but contains ‘ghost cells’ and spherical calcifications.
The so-called calcifying odontogenic cyst (COC) represents
a heterogeneous group of lesions that exhibit a variety of
clinicopathologic and behavioral features. Because of this
diversity, there has been confusion and disagreement on the
terminology and classification of these lesions. The term
calcifying odontogenic cyst includes both non-neoplastic
cyst and true neoplasms. The 1992 WHO classification
includes this cyst and all its variants in the category of
odontogenic tumors.
It has become obvious that it is not one lesion but really
two—a cystic lesion and a solid neoplastic lesion. A third
malignant counterpart of the neoplastic lesion may be added.
The calcifying odontogenic cyst may present as an intraosseous
cystic lesion or in association with an odontoma. On rare
occasions, it may present as a peripheral (gingival) lesion.
The neoplasm, more aptly termed a dentinogenic ghost
cell tumor, is a rare lesion that may occur in bone or in the
gingival soft tissue (for detailed description refer section on
odontogenic tumors).
Glandular Odontogenic Cyst
(Sialo-odontogenic cyst, mucoepidermoid odontogenic cyst)
An unusually large solitary or multilocular odontogenic cyst
probably derived from the rests of dental lamina, consisting
of a stratified squamous epithelium containing numerous
mucus-secreting cells.
The glandular odontogenic cyst (GOC), also known as
sialo-odontogenic cyst has many similarities to the lateral
periodontal cyst of which it is considered a variant by some
authors. Glandular odontogenic cyst occurs in the same
location as the lateral periodontal cyst but as a rule it has a
SECTION I
multilocular radiolucent appearance. The name of the cyst is
not yet established. The term most descriptive of the lesion is
probably mucoepidermoid odontogenic cyst because of the
presence of both secretory elements and stratified squamous
Chapter4.indd 272
epithelium (Sadeghi et al, 1991). The use of this name
might, however, lead to confusion with the mucoepidermoid
carcinoma, and is therefore unlikely to find favor (Shear, 1992).
Padayachee and van Wyk, 1987, indicated that the typical
features of the cyst are that it is intrabony and multilocular
radiographically; that it can recur if not adequately excised; that
it is multicystic, with the cystic spaces lined by a nonkeratinized
epithelium similar to reduced enamel epithelium, with
epithelial thickenings or plaques; that mucous and cylindrical
cells form an integral part of the epithelial component; and
that mucinous material within the cystic spaces is a prominent
feature. Gardner et al, (1988) who favored the name ‘glandular
odontogenic cyst’ suggested that its histological features and
biological behavior are sufficient for it to be regarded as an
entity.
Clinical Features. The glandular odontogenic cyst occurs
over a wide age range. A slight male predilection was reported.
The common site affected was anterior mandible. The
lesions showed slow progressive growth, were painless and
locally destructive. The mandible seems to be affected more
commonly (87.2%) than the maxilla. The age ranges from
10–90 years with the mean of 49.5 years.
Radiographic Features. The lesions appear well defined
with a multilocular pattern but without specific diagnostic
features.
Histologic Features. Histologically, glandular odontogenic
cyst is lined in parts by a nonkeratinized stratified epithelium of
varying thickness (Fig. 4-14). The epithelium has a glandular
or pseudoglandular structure, with goblet mucous producing
cells as well as intraepithelial crypts or microcysts containing
mucus. These microcysts may open onto the surface of the
epithelium giving a papillary or corrugated appearance.
Some cells may also be ciliated. Occasionally the epithelium
is thinner, similar to reduced enamel epithelium. Epithelial
thickenings or plaques may be present either in this thin
epithelium or in the stratified epithelium. Interface between
epithelium and connective tissue is flat. The diagnosis of
Figure 4-14. Sialo-odontogenic cyst.
Nonkeratinized stratified epithelium of varying thickness; cuboidal or columnar
ciliated surface lining with goblet cells; polycystic with secretory and epithelial
elements; microcysts may form duct like structures and are filled with a PAS-
positive material surrounded by fibrous capsule.
01/06/12 10:54 AM

glandular odontogenic cyst should be considered when
observing a lateral periodontal multilocular radiolucency. The
diagnosis is essentially microscopic.
Treatment. The treatment should be conservative but with a
careful dissection of the margins in order to avoid recurrences.
Patient should be followed-up periodically in order to assess
early recurrences.
INFLAMMATORY CYSTS
Periapical Cyst
(Radicular cyst, apical periodontal cyst, root end cyst)
The periapical (radicular) cyst is the most common odontogenic
cyst. The usual etiology is an infected tooth, leading to necrosis
of the pulp. Toxins exit at the apex of the tooth, leading to
periapical inflammation. This inflammation stimulates the
epithelial rests of Malassez, which are found in the apical
periodontal ligament, resulting in the formation of a periapical
granuloma that may be infected or sterile. Eventually, this
epithelium undergoes necrosis caused by a lack of blood
supply, and the granuloma becomes a cyst (periapical cyst).
The lesions are not usually clinically detectable when small
but most often are discovered as an incidental findings on
radiographic survey.
Pathogenesis. This cyst is classified as inflammatory,
because in the majority of cases it is a consequence to pulpal
necrosis following caries, with an associated periapical
inflammatory response. Other causes include any event
that may result in pulpal necrosis such as tooth fracture and
improper restorations, among others. The first line of defense
to pulpal necrosis in the periapical area is the formation of
a granuloma. A granuloma is a highly vascularized tissue
containing a profuse infiltrate of immunologically competent
cells, i.e. lymphocytes, macrophages and plasma cells.
The epithelial rests of Malassez, which are pluripotential in
nature can differentiate into any type of epithelium, under the
proper stimuli. These rests play a central role in the formation
of radicular cysts. In the midst of the rich vascular area provided
by the periapical granuloma, the rests of Malassez proliferate and
eventually form a large mass of cells. With continuous growth,
the inner cells of the mass are deprived of nourishment and they
undergo liquefaction necrosis. This leads to the formation of a
cavity which is located in the center of the granuloma, giving
rise to a radicular cyst.
Islands of squamous epithelium which have developed from
odontogenic rests of Malassez can also be found in a periapical
granuloma without cystic transformation. Endodontists refer
to these granulomas as ‘bay cyst’.
Clinical Features. Around 60% of all jaw cysts are radicular
or residual cysts. Radicular cysts can occur in the periapical area
of any teeth, at any age but are seldom seen associated with the
primary dentition. The majority of cases of apical periodontal
cysts are asymptomatic. The tooth is seldom painful or even
sensitive to percussion. This type of cyst is only infrequently
of such a size that it destroys much bone, and even more
Chapter4.indd 273
273
Cysts and Tumors of Odontogenic Origin
rarely does it produce expansion of the cortical plates. The
apical periodontal cyst is a lesion that represents a chronic
inflammatory process and develops only over a prolonged
period of time. In some cases, such a cyst of long standing may
undergo an acute exacerbation of the inflammatory process
and develop rapidly into an abscess (periapical abscess) that
may then proceed to a cellulitis or form a draining fistula. The
cause of such a sudden flare up is not known, but it may be a
result of loss of local or generalized tissue resistance.
Radiographic Features. The radiographic image of the ra-
dicular cyst is a peri- or para-apical, round or oval radiolucen-
cy of variable size which is generally well delineated and most
likely with a marked radiopaque rim. Other lesions, such as
granulomas, neoplasms of various origin and some diseases
of bone can also present a similar radiolucent periapical ap-
pearance. Therefore, a periapical radiolucency cannot be auto-
matically assumed to be a cyst. Several studies have indicated
that it is not possible to rely on the radiographic size of a peri-
apical radiolucency to establish the diagnosis of either cyst or
granuloma unless the lesion is larger than 2 cm in diameter.
Rarely radicular cysts will induce resorption of the root of the
affected tooth.
Histologic Features. Microscopically a radicular cyst is lim-
ited by a mature collagenous connective tissue wall. Abundant
fibroblasts can be identified within the cystic wall. The wall
generally presents an inflammatory infiltrate of variable de-
gree. Lymphocytes are generally the most prominent cells in
the infiltrate and are characterized by their darkly stained nu-
cleus, which occupies most of the cytoplasm. Plasma cells are
also abundant in cysts’ walls and mostly seen in long standing
(chronic) cysts. They are characterized by an eccentric nucle-
us with a cartwheel arrangement of the nuclear chromatin.
Plasma cells are considered repertoire of immunoglobulins.
Other histological findings within the cystic wall are: erythro-
cytes and areas of hemorrhage, occasional spicules of dystro-
phic bone, multinucleated giant cells and cholesterol crystals
(Fig. 4-15A).
The cavity of a radicular cyst is generally lined by stratified
squamous epithelium. These cysts can be lined by respiratory
epithelium, especially if they are in the vicinity of the maxillary
sinus. The epithelial lining, many times, is discontinuous,
frequently missing over areas of intense inflammation. Rarely
radicular cysts may be lined by mucus producing epithelium
in either maxillary or mandibular locations. The mucous
epithelium is the result of metaplastic transformation of the
epithelial rests of Malassez which are pluripotential. In rare
instances, carcinoma has been reported developing from the
lining epithelium of odontogenic cysts, including the radicular
cyst. These have been reviewed by Gardner (1969).
An interesting and peculiar structure, originally described
by Rushton and subsequently reported by Molyneux, Medak
and Weinmann and Shear, is the hyaline body or Rushton
body, often found in great numbers in the epithelium of
SECTION I
apical, periodontal or residual cysts (Fig. 4-15 B). These
hyaline bodies are tiny linear or arc-shaped bodies, generally
associated with the lining epithelium, that appear amorphous
01/06/12 10:54 AM
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Disturbances of Development and Growth
A of patients. They may have been present for many months or
B This cyst is of rare occurrence and must be radiographically
Figure 4-15. Radicular cyst.
(A) The cyst cavity is filled with an eosinophilic coagulum containing slit-like
spaces which contain cholesterol. These cholesterols may form mucosal nodules;
the epithelial lining becomes discontinued where such nodules have formed. (B)
Hyaline bodies. Another feature that may be seen within the epithelial linings
of radicular cyst is the peculiarly shaped structures which are eosinophilic, and
typically, are described as lady’s hairpin shaped (Courtesy of Dr K W Lee).
in structure, eosinophilic in reaction and brittle in nature,
since they evidence fracture in some cases. Their frequency of
occurrence in cyst linings ranges between 2.6 and 9.5% of cysts,
according to a review by Allison. The etiology, pathogenesis,
and significance of these structures are unknown. The lumen
of the cyst usually contains a fluid with a low concentration of
protein that stains palely eosinophilic. Occasionally the lumen
may contain a great deal of cholesterol, and in rare instances,
limited amounts of keratin are present.
Treatment. The treatment of the radicular cyst consists of
extraction of the involved teeth and careful curettage of the
periapical tissue. Under some conditions, root canal therapy
may be carried out with apicoectomy of the cystic lesion.
The cyst does not recur if surgical removal is thorough. If the
cystic sac is badly fragmented, leaving epithelial remnants,
or if a periapical granuloma is incompletely removed with
epithelial rests remaining, a residual cyst may develop in this
area months or even years later. If untreated, the radicular
SECTION I
cyst slowly increases in size at the expense of the surrounding
bone. The bone undergoes resorption, but seldom is there a
remarkable expansion of the cortical plates, as is frequently
seen in the case of the dentigerous cyst.
Chapter4.indd 274
Residual Cyst
Residual cyst is a term of convenience because no teeth are
left by which to identify the lesion. Most commonly, these
actually are retained periapical cysts from teeth that have been
removed. The histology of the lining is a nondescript stratified
squamous epithelium.
Theoretically, it could develop in a dental granuloma that is left
after an extraction. The residual cyst may be found in any of the
tooth bearing areas of the mandible or maxilla. Morphologically,
the residual cyst may present as a fairly well defined radiolucency
that can vary in size from a few mm to several cm. Clinically,
these cysts are usually found on routine radiographic examination
years and only become symptomatic upon secondarily infected
(Figs 4-16 A, B, and C). Usually, residual cysts do not expand
bone. Treatment is surgical curettage (refer periapical cyst).
Paradental Cyst
A cyst of uncertain origin found primarily on the distal or
facial aspect of a vital mandibular third molar, consisting of
intensely inflamed connective tissue and epithelial lining.
The paradental cyst is an inflammatory cyst which develops
on the lateral surface of a tooth root. Some authors refer to
this cyst as an inflammatory periodontal cyst or collateral cyst.
differentiated from the lateral periodontal cyst. It is treated
by surgical ablation and does not have a tendency to recur.
It seems clear that the paradental cyst is of inflammatory origin
and that it arises from odontogenic epithelium. Craig (1976) has
suggested that either the cell rests of Malassez or the reduced
enamel epithelium might provide the cell of origin. He favored
the latter source, arguing that in his study the rests of Malassez
always appeared inactive and that if the Malassez rests were
responsible the lesions should be equally distributed around the
root surface. His serial sections indicated that the development
of paradental cyst may follow hyperplasia and cystic change in
reduced enamel epithelium. He suggested that the presence of
an extension of reduced enamel epithelium over the enamel
projections might be the source, and could explain the frequent
buccal location of the cyst.
Pathogenesis. There is no unanimity with regard to patho-
genesis. Ackerman, Cohen and Altini (1987) like Craig (1976),
favored origin from reduced enamel epithelium but suggested
that cyst formation occurs as a result of unilateral expansion of
the dental follicle secondary to inflammatory destruction of peri-
odontium and the alveolar bone. Fowler and Brannon (1989)
suggested that it may be a variant of the dentigerous cyst or de-
rived from an occluded periodontal pocket. Vedtofte and Praeto-
rius (1989) were satisfied that the cyst was of inflammatory ori-
gin, initiated by a pericoronitis at the time of tooth eruption and
considered rests of Malassez and reduced enamel epithelium the
most likely source of the cyst epithelium.
Clinical Features. In the sample of 2,616 jaw cysts reported
by Shear (1992), there were 65 paradental cysts classified over
a 32-year period (2.5%). The series of 50 cases reported by
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A
B odontogenic epithelia and/or ectomesenchyme with variable
C
Figure 4-16. Residual cyst.
Histopathological features of (A), residual cyst using H & E stain (x 10), (B) of
residual cyst using PCNA antibody stain (x 10), and (C) of residual cyst using
PCNA antibody stain (x 40).
Ackerman, Cohen and Altini (1987) represented the 3% of
a sample of 1,852 odontogenic cysts observed over a 20-year
period. Virtually all the cases in the study by Ackermann,
Cohen and Altini occurred between the ages of 10 and 39
with two-thirds of their sample in the third decade; the same
as in Craig’s material (cited by Shear, 1992). There was a
considerable preponderance of males reported by Ackermann,
Cohen and Altini and by Fowler and Brannon but of Vedtofte
and Praetorius. There was an equal gender distribution.
Chapter4.indd 275
275
Cysts and Tumors of Odontogenic Origin
Ackermann, Cohen and Altini found most of their cyst
located distally and distobuccally to the third molar. All the
papers emphasized that the involved teeth were vital. Bilateral
examples occurred in a number of instances.
Radiographic Features. All authors reported a variable
radiographic picture but there are some features which appeared
consistently and which seem to be useful in contributing to
the diagnosis. These are the non widening of the periodontal
ligament space and that the lesion was superimposed on the
buccal root face. When there was a distal as well as a buccal
radiolucency, the distal element was separate from the distinct
distal follicular space (Shear, 1992).
Histologic Features. Histologically, the cysts were lined by
a hyperplastic nonkeratinized stratified squamous epithelium.
An intense inflammatory cell infiltrate is present associated
with the hyperplastic epithelium and in the fibrous capsule
adjacent to the epithelium. Histologically the paradental cyst
cannot be differentiated from a radicular cyst.
Treatment. Enucleation and extraction of associated molar.
TUMORS OF ODONTOGENIC ORIGIN
Odontogenic tumors represent a spectrum of lesions ranging
from malignant (rare) and benign neoplasms to dental
hamartomas, all arising from odontogenic residues, i.e.
amounts of dental hard tissues formed generally in the same
sequence as in normal tooth development.
Occasionally an odontogenic tumor develops from a preexisting
developmental cyst (e.g. adenomatoid odontogenic tumor from
a dentigerous cyst) or dental primordium (e.g. ameloblastomas
often take the place of lower third molars). In many instances
the exact tissue of origin (histogenesis) of an odontogenic tumor
may only be inferred from its site and structure.
Classification of Odontogenic Tumors. The WHO clas-
sification (1992) divides odontogenic tumors into benign and
malignant, with major subdivisions in each category (White,
2004) (Table 4-2). The subdivisions for the benign neoplasms
are based on types of odontogenic tissue involved in the pro-
cess and include:
O Odontogenic epithelium without odontogenic ectomesen-
chyme.
O Odontogenic epithelium with odontogenic ectomesen-
chyme, with or without dental hard tissue formation.
O Odontogenic ectomesenchyme with or without included
odontogenic epithelium.
The classic example of odontogenic tumor viz. amelo-
blastoma is an archetype of a neoplasm where the neoplastic
component is epithelial only without contribution from the
ectomesenchyme. In the second category, some of the neo-
plasms are pure epithelial tumors but are capable of inducing
SECTION I
dysplastic enamel and dentin formation. Examples include
adenomatoid odontogenic tumor, calcifying odontogenic cyst,
and odontoameloblastoma. Other tumors in the category
exhibit neoplastic cells of both odontogenic epithelium and
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