642
C H A P T E R 1 5 Odontogenic Cysts and Tumors
• Fig. 15-25  Nevoid Basal Cell Carcinoma Syndrome. Plantar
pits.
• Fig. 15-26  Nevoid Basal Cell Carcinoma Syndrome. Chest film
showing presence of bifid ribs (arrows).
initial presentation, but in most cases additional cysts will
develop over periods ranging from 1 to 20 years.
Radiographically, the cysts in patients with nevoid basal
cell carcinoma syndrome do not differ significantly from
isolated OKCs. The cysts in patients with this syndrome are
often associated with the crowns of unerupted teeth; on
radiographs they may mimic dentigerous cysts (Fig. 15-28).
Diagnostic criteria for the nevoid basal cell carcinoma
syndrome are provided in Box 15-3.
Histopathologic Features
The cysts in the nevoid basal cell carcinoma syndrome
histopathologically are invariably OKCs. The keratocysts
in patients with this syndrome tend to have more sat­
ellite cysts, solid islands of epithelial proliferation, and
• Fig. 15-27  Nevoid Basal Cell Carcinoma Syndrome. Antero-
posterior skull film showing calcification of the falx cerebri. (Courtesy
of Dr. Ramesh Narang.)
odontogenic epithelial rests within the fibrous capsule
than do isolated keratocysts (Fig. 15-29). Foci of calci-
fication also appear to be more common. These features,
however, are not diagnostic for nevoid basal cell carcinoma
syndrome because they may be seen in isolated kerato-
cysts. OKCs associated with this syndrome have been
shown to demonstrate overexpression of p53 and cyclin
D1 (bcl-1) oncoproteins when compared with nonsyn-
drome keratocysts.
The basal cell tumors of the skin cannot be distinguished
from ordinary basal cell carcinomas. They exhibit a wide
spectrum of histopathologic findings, from superficial
basal cell lesions to aggressive, noduloulcerative basal cell
carcinomas.
Treatment and Prognosis
Most of the anomalies in nevoid basal cell carcinoma syn-
drome are minor and usually not life threatening. The
prognosis generally depends on the behavior of the skin
tumors. In a few cases, aggressive basal cell carcinomas
have caused the death of the patient as a result of tumor
invasion of the brain or other vital structures (Figs. 15-30
and 15-31). Because the development of the basal cell
carcinomas seems to be triggered by ultraviolet (UV) light
exposure, patients should take appropriate precautions to
avoid sunlight. For the same reason, radiation therapy
should be avoided if at all possible. The jaw cysts are
treated in the same manner as isolated OKCs, but in many
patients additional cysts will continue to develop. Varying
degrees of jaw deformity may result from the operations
for multiple cysts. Infection of the cysts in patients with
 CHAPTER 15  Odontogenic Cysts and Tumors 643

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

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

• Fig. 15-29  Nevoid Basal Cell Carcinoma Syndrome. Odonto-
genic keratocyst (OKC) showing numerous odontogenic epithelial rests
in the cyst wall.
• Fig. 15-30  Nevoid Basal Cell Carcinoma Syndrome. This
52-year-old man had more than 100 basal cell carcinomas removed
from his face over a 30-year period. Several basal cell carcinomas are
present in this photograph. The lesion at the inner canthus of the left
eye was deeply invasive and was eventually fatal as a result of brain
invasion.
644 C H A P T E R 1 5 Odontogenic Cysts and Tumors
• Fig. 15-31  Nevoid Basal Cell Carcinoma Syndrome. Facial
deformity secondary to multiple surgical procedures to remove basal
cell carcinomas.
this syndrome is also relatively common. Some investigators
have suggested that affected children should have magnetic
resonance imaging (MRI) studies every 6 months until 7
years of age to monitor for the development of medullo-
blastoma. Genetic counseling is appropriate for affected
individuals.
◆GINGIVAL (ALVEOLAR) CYST
OF THE NEWBORN
Gingival cysts of the newborn are small, superficial,
keratin-filled cysts that are found on the alveolar mucosa of
infants. These cysts arise from remnants of the dental
lamina. They are common lesions, having been reported in
up to half of all newborns. However, because they disappear
spontaneously by rupture into the oral cavity, the lesions
seldom are noticed or sampled for biopsy. Similar inclusion
cysts (e.g., Epstein’s pearls and Bohn’s nodules) are also
found in the midline of the palate or laterally on the hard
and soft palate (see page 24).
Clinical Features
Gingival cysts of the newborn appear as small, usually mul-
tiple whitish papules on the mucosa overlying the alveolar
processes of neonates (Fig. 15-32). The individual cysts are
• Fig. 15-32  Gingival Cyst of the Newborn. Multiple whitish
papules on the alveolar ridge of a newborn infant.
usually no more than 2 to 3 mm in diameter. The maxillary
alveolus is more commonly involved than the mandibular.
Histopathologic Features
Examination of an intact gingival cyst of the newborn
shows a thin, flattened epithelial lining with a parakeratotic
luminal surface. The lumen contains keratinaceous debris.
Treatment and Prognosis
No treatment is indicated for gingival cysts of the newborn
because the lesions spontaneously involute as a result of the
rupture of the cysts and resultant contact with the oral
mucosal surface. The lesions are rarely seen after 3 months
of age.
◆ GINGIVAL CYST OF THE ADULT
The gingival cyst of the adult is an uncommon lesion. It
is considered to represent the soft tissue counterpart of the
lateral periodontal cyst (see next topic), being derived
from rests of the dental lamina (rests of Serres). The diag-
nosis of gingival cyst of the adult should be restricted to
lesions with the same histopathologic features as those of
the lateral periodontal cyst. On rare occasions, a cyst may
develop in the gingiva at the site of a gingival graft; however,
such lesions probably represent epithelial inclusion cysts that
are a result of the surgical procedure.
Clinical Features
Like the lateral periodontal cyst, the gingival cyst of the
adult shows a striking predilection to occur in the mandibu-
lar canine and premolar area (60% to 75% of cases). Gin-
gival cysts of the adult are most commonly found in patients
in the fifth and sixth decades of life. They are almost invari-
ably located on the facial gingiva or alveolar mucosa. Maxil-
lary gingival cysts are usually found in the incisor, canine,
and premolar areas.

• Fig. 15-33  Gingival Cyst of the Adult. Tense, fluid-filled swelling
on the facial gingiva.
• Fig. 15-34  Gingival Cyst of the Adult. Low-power photomicro-
graph showing a thin-walled cyst in the gingival soft tissue.
Clinically, the cysts appear as painless, domelike swell-
ings, usually less than 0.5 cm in diameter, although rarely
they may be somewhat larger (Fig. 15-33). They are often
bluish or blue-gray. In some instances, the cyst may cause
a superficial “cupping out” of the alveolar bone, which is
usually not detected on a radiograph but is apparent when
the cyst is excised. If more bone is missing, one could argue
that the lesion may be a lateral periodontal cyst that has
eroded the cortical bone rather than a gingival cyst that
originated in the mucosa.
Histopathologic Features
The histopathologic features of the gingival cyst of the adult
are similar to those of the lateral periodontal cyst, consisting
of a thin, flattened epithelial lining with or without focal
plaques that contain clear cells (Figs. 15-34 and 15-35).
Small nests of these glycogen-rich clear cells, which repre-
sent rests of the dental lamina, also may be seen in the
surrounding connective tissue. Sometimes the cystic lining
is so thin that it is easily mistaken for the endothelial lining
of a dilated blood vessel.
CHAPTER 15  Odontogenic Cysts and Tumors 645
• Fig. 15-35  Gingival Cyst of the Adult. High-power photomicro-
graph showing a plaquelike thickening of the epithelial lining.
Treatment and Prognosis
The gingival cyst of the adult responds well to simple surgi-
cal excision. The prognosis is excellent.
◆ LATERAL PERIODONTAL CYST
(BOTRYOID ODONTOGENIC CYST)
The lateral periodontal cyst is an uncommon type of
developmental odontogenic cyst that typically occurs along
the lateral root surface of a tooth. It is believed to arise from
rests of the dental lamina, and it represents the intrabony
counterpart of the gingival cyst of the adult. The lateral
periodontal cyst accounts for less than 2% of all epithelium-
lined jaw cysts.
In the past, the term lateral periodontal cyst was used to
describe any cyst that developed along the lateral root
surface, including lateral radicular cysts (see page 120) and
OKCs (see page 636). However, the lateral periodontal cyst
has distinctive clinical and microscopic features that distin-
guish it from other lesions that sometimes develop in the
same location.
Clinical and Radiographic Features
The lateral periodontal cyst is most often an asymptomatic
lesion that is detected only during a radiographic examina-
tion. It most frequently occurs in patients in the fifth
through the seventh decades of life; rarely does it occur in
someone younger than age 30. Around 75% to 80% of
cases occur in the mandibular premolar-canine-lateral
incisor area. Maxillary examples also usually involve this
same tooth region (Fig. 15-36).
Radiographically, the cyst usually appears as a well-
circumscribed radiolucent area located laterally to the root
or roots of vital teeth. Most such cysts are less than 1.0 cm
in greatest diameter (Figs. 15-37 and 15-38). In rare
instances, multifocal lateral periodontal cysts have been
described. In addition, some examples can develop in eden-
tulous sites.
646 C H A P T E R 1 5 Odontogenic Cysts and Tumors
1% 15% 4%
1% 66% 14%
• Fig. 15-36  Lateral Periodontal Cyst. Relative distribution of
lateral periodontal cysts in the jaws.
• Fig. 15-37  Lateral Periodontal Cyst. Radiolucent lesion between
the roots of a vital mandibular canine and first premolar.
Occasionally, the lesion may have a polycystic appear-
ance; such examples have been termed botryoid odonto-
genic cysts. Grossly and microscopically, they show a
grapelike cluster of small individual cysts (Fig. 15-39).
These lesions are generally considered to represent a variant
of the lateral periodontal cyst, possibly the result of cystic
degeneration and subsequent fusion of adjacent foci of
dental lamina rests. The botryoid variant often shows a
multilocular radiographic appearance, but it also may
appear unilocular.
The radiographic features of the lateral periodontal cyst
are not diagnostic; an OKC that develops between the roots
of adjacent teeth may show identical radiographic findings.
An inflammatory radicular cyst that occurs laterally to a
root in relation to an accessory foramen or a cyst that arises
from periodontal inflammation also may simulate a lateral
• Fig. 15-38  Lateral Periodontal Cyst. A larger lesion causing root
divergence.
• Fig. 15-39  Lateral Periodontal Cyst. Gross specimen of a bot-
ryoid variant. Microscopically, this grapelike cluster revealed three
separate cavities.
periodontal cyst radiographically (see page 120). In one
study of 46 cases of cystic lesions in the lateral periodontal
region, only 13 met the histopathologic criteria for the
lateral periodontal cyst; eight were OKCs, 20 were inflam-
matory cysts, and five were of undetermined origin.
Histopathologic Features
The lateral periodontal cyst has a thin, generally nonin-
flamed, fibrous wall, with an epithelial lining that is only
one to three cells thick in most areas. This epithelium
usually consists of flattened squamous cells, but sometimes
the cells are cuboidal in shape. Foci of glycogen-rich clear
cells may be interspersed among the lining epithelial cells.
Some cysts show focal nodular thickenings of the lining

B 1. Calcifying cystic odontogenic tumor
• Fig. 15-40  Lateral Periodontal Cyst. A, This photomicrograph
shows a thin epithelial lining with focal nodular thickenings. B, These
thickenings often show a swirling appearance of the cells.
epithelium, which are composed chiefly of clear cells (Fig.
15-40). Clear cell epithelial rests sometimes are seen within
the fibrous wall. Rarely, lateral periodontal cysts exhibit
focal areas that histopathologically are suggestive of the
glandular odontogenic cyst (see page 649).
Treatment and Prognosis
Conservative enucleation of the lateral periodontal cyst is
the treatment of choice. Usually, this can be accomplished
without damage to the adjacent teeth. Recurrence is unusual,
although it has been reported with the botryoid variant,
presumably because of its polycystic nature. An exceedingly
rare case of squamous cell carcinoma, which apparently
originated in a lateral periodontal cyst, also has been
reported.
◆ CALCIFYING ODONTOGENIC CYST
(CALCIFYING CYSTIC ODONTOGENIC
TUMOR; GORLIN CYST; DENTINOGENIC
GHOST CELL TUMOR; GHOST CELL
ODONTOGENIC CARCINOMA)
First described in 1962 by Gorlin and associates, the calci-
fying odontogenic cyst is part of a spectrum of lesions
CHAPTER 15  Odontogenic Cysts and Tumors 647
2% 12% 38%
11% 10% 27%
• Fig. 15-41  Calcifying Odontogenic Cyst. Relative distribution of
calcifying odontogenic cysts in the jaws.
characterized by odontogenic epithelium containing “ghost
cells,” which then may undergo calcification. Although
most examples grow in a cystic fashion, some lesions
occur as solid tumorlike growths (dentinogenic ghost cell
tumor). Therefore, in the current WHO classification
system, these lesions have been categorized as odontogenic
tumors under three categories (based on the cystic, solid, or
malignant nature of the lesion):
2. Dentinogenic ghost cell tumor
3. Ghost cell odontogenic carcinoma
The overwhelming majority of intraosseous ghost cell
odontogenic lesions grow as cystic lesions, and less than 5%
of cases can be classified as solid dentinogenic ghost cell
tumors. Therefore, the authors still prefer to use the simpler
designation “calcifying odontogenic cyst” for the cystic
examples. Approximately one-third of peripheral lesions
will be solid in nature, although these peripheral examples
are not as aggressive as their intraosseous counterparts.
The calcifying odontogenic cyst may be associated with
other recognized odontogenic tumors, most commonly
odontomas. However, adenomatoid odontogenic tumors
and ameloblastomas have also been associated with calcify-
ing odontogenic cysts.
Clinical and Radiographic Features
Intraosseous calcifying odontogenic cysts occur with about
equal frequency in the maxilla and mandible. About 65%
of cases are found in the incisor and canine areas (Fig.
15-41). The mean age is 30 years, and most cases are diag-
nosed in the second to fourth decades of life. Calcifying
odontogenic cysts that are associated with odontomas tend
to occur in younger patients, with a mean age of 17 years.
The central calcifying odontogenic cyst is usually a uni-
locular, well-defined radiolucency, although the lesion occa-
sionally may appear multilocular. Radiopaque structures
within the lesion, either irregular calcifications or toothlike
densities, are present in about one-third to one-half of cases
(Fig. 15-42). In approximately one-third of cases, the radio-
lucent lesion is associated with an unerupted tooth, most