11-Odontogenic Tumours 2

BENIGN EPITHELIAL
ODONTOGENIC TUMOURS
2. Calcifying Epithelial Odontogenic Tumour (CEOT)
CALCIFYING
EPITHELIAL
ODONTOGENIC TUMOR
PINDBORG TUMOR
CALCIFYING EPITHELIAL ODONTOGENIC
TUMOR
 “ A benign, locally aggressive tumor consisting of strands and

medullary patterns of squamous and clear cells that are often
accompanied by spherical calcifications and amyloid staining
hyaline deposits”
 Less than 1% of all odontogenic tumors.
 Central (Intraosseous) or Peripheral (Extra-osseous) forms

CALCIFYING EPITHELIAL ODONTOGENIC TUMOR
PATHOGENESIS/ETIOLOGY
 Histogenesis is uncertain.
 Dental lamina remnants (anatomic distribution in the jaws)
 REE overlying the tooth crown
 The stratum intermedium of the enamel organ (resemblance to cells)

CLINICAL FEATURES
 Wide age range, most often between 30 and 50 years of

age.
 No gender predilection.
 2/3rd cases in the mandible, mostly posterior areas (molar >

premolar)
 Clinically presents as a painless, slow growing swelling
 Nasal obstruction, epistaxis and proptosis in maxilla

CLINICAL FEATURES
 Peripheral (extraosseous) calcifying epithelial odontogenic tumor

appear as nonspecific, sessile gingival masses, most often on the
anterior gingiva (both in tooth bearing and edentulous area)
CLINICAL FEATURES
CLINICAL FEATURES
CLINICAL FEATURES
RADIOGRAPHIC FEATURES
 Unilocular or a multilocular radiolucent defect (unilocular pattern

commonly in the maxilla)
 May be entirely radiolucent, but usually contains calcified structures of

varying size and density.
 Calcifications are usually scattered within the tumor or are prominent

around the crown of the impacted tooth (12%)
 Driven snow pattern is noted less frequently

 Well-defined scalloped margins,
 20% of cases have an ill-defined periphery,
 Additional 20% exhibit a corticated border.
 Frequently associated with an impacted tooth, most often a mandibular

molar.
 Peripheral (extraosseous) calcifying epithelial odontogenic tumor is RL

with cupped-out erosion of the underlying bone.
HISTOPATHOLOGIC FEATURES
 EPITHELIAL COMPONENT
 Discrete islands, strands, or sheets of polyhedral epithelial
cells(squamous) in a fibrous stroma
 Cellular outlines of the epithelial cells are distinct, and intercellular

bridges may be noted.
 Nuclear pleomorphism seen but not considered to indicate malignancy.

 EPITHELIAL COMPONENT
 Clear cell variant: clear cells constitute a significant portion of the
epithelial component in lobular pattern , less tendency to form amyloid
or calcifications
A: sheet of atypical epithelial cells, B: amyloid droplets with calcific dystrophic calcifications
 STROMAL COMPONENT
 1. Amyloid
 Large areas of amorphous, eosinophilic, hyalinized (amyloid-

like) extracellular material
 The tumor islands frequently enclose masses of this hyaline

material; resulting in a cribriform appearance.
 STROMAL COMPONENT
 2. Calcifications
 Develop within the amyloid-like material
 Form concentric rings (liesegang ring calcifications)
 These tend to fuse and form large, complex masses.

 AMYLOID
 The amyloid-like material in the pindborg tumor stains as

amyloid.
 Positive staining results with CONGO RED OR THIOflAVINE T
 APPLE-GREEN BIREFRINGENCE after congo red

staining when viewed with polarized light.
CEOT
CEOT
 VARIATIONS
 Some tumors consist of large sheets of epithelial cells with
minimal production of amyloid-like material and calcifications.
 Some have large diffuse masses of amyloid-like material and

contain only small nests or islands of epithelium.
TREATMENT AND PROGNOSIS
 Originally believed that the CEOT had the same biologic behavior as the ameloblastoma,
accumulating experience indicates that it is less aggressive.
 Conservative local resection to include a narrow rim of surrounding bone appears to be the
treatment of choice,
 lesions in the posterior maxilla should probably be treated more aggressively.
 A recurrence rate of about 15% has been reported;
 The overall prognosis appears good,
 Rare examples of malignant or borderline malignant calcifying epithelial odontogenic tumor

have been reported, with documented metastasis to regional lymph nodes and lung.
BENIGN ODONTOGENIC
TUMOURS
3. ADENOMATOID ODONTOGENIC TUMOR

ADENOMATOID
ODONTOGENIC TUMOR
A well circumscribed lesion derived from odontogenic epithelium that
usually occurs around the crown of un-erupted anterior teeth of young
patient and consists of epithelium in swirls and ductal patterns
interspersed with spherical calcifications

ADENOMATOID ODONTOGENIC TUMOR
 3% to 7% of all odontogenic tumors
 Enamel organ epithelium,
 Remnants of dental lamina.

CLINICAL FEATURES
 Younger patients, mostly 10 to 19  Small, rarely > 3cm in D.
years of age.  Asymptomatic
 Striking tendency for ANTERIOR  Large lesions may cause painless

PORTIONS of the jaws expansion of bone
 Twice as often in the MAXILLA as 
Rarely, Peripheral (extraosseous)
in the mandible AOT appear as small, sessile masses
 Females > males. on the facial gingiva of the maxilla.
 FOLLICUAR TYPE:
 Circumscribed, unilocular radiolucency involving the crown of an

unerupted tooth, most often a canine.
 RL sometimes extends apically along the root past the CEJ

 EXTRA-FOLLICULAR TYPE
 Well-delineated unilocular radiolucency not related to an unerupted

tooth, located between the roots of erupted teeth
 May be completely radiolucent; often contains fine (snowflake)

calcifications
 well-defined lesion usually surrounded by a thick, fibrous capsule.
 central portion of the tumor may be essentially solid or may show
varying degrees of cystic change
EPITHELIAL STRUCTURES/CELLS
 1. Spindle Shaped Epithelial Cells that form

 Sheets
 Strands, or
 Whorled masses in a scant fibrous stroma.

(a) Cellular multinodular proliferation of cells in the form of nests and rosettes. (b) Solid areas of cells in the form of whorls interspersed with areas of calcification. (c) Duct-like pattern lined
by cuboidal cells, inset showing the characteristic rosette like structure (d) Curved rows of cells giving a tubular appearance
EPITHELIAL STRUCTURES/CELLS
 2. Rosette-like Structures
 About a central space, may be empty or contain small
amounts of eosinophilic material.
 This material may stain for amyloid.

(a) Convoluted structure of tall columnar epithelial cells (b) Strands of tumor cells 1 to 2 cell layer thick at the periphery (c) Thin anastamosing strands of basloid cells in a plexiform pattern Inset
showing high power view of the same (d) Cellular areas with cribriform configuration
EPITHELIAL STRUCTURES/CELLS 3. Tubular or Duct-like Structures
 Characteristic feature, may be prominent, scanty, or even absent
 Consist of a central space surrounded by a layer of columnar or cuboidal epithelial
cells.
 The nuclei of these cells tend to be polarized away from the central space.
 Likely the result of the secretory activity of the tumor cells, which appear to be
pre-ameloblasts.
 These structures are not true ducts, and no glandular elements are present in the
tumor
 Rather are microcysts
 Sometimes lumen of these structures is lined by an eosinophilic rim called as hyaline ring
The odontogenic cells constituted of whorled nests of epithelium together with areas of glandular or ductal pattern
Microscopic photograph showing tumor nodule composed of spindle-shaped or cuboidal epithelial cells forming rosette-like structures
Photomicrograph showing solid nodules of cuboidal epithelium arranged in sheets, rosettes (white arrow),
and duct like areas (black arrow), together with focal areas of basophilic calcification (red arrow)
Microscopic photograph showing duct-like structures of odontogenic epithelium lined by a single row of cuboidal or low columnar epithelial cells
Characteristic duct like structures comprised a central lumen lined by tall columnar cells in a single layer scattered throughout the tumor
 STROMAL COMPONENTS
Loose, hypocellular or Fibrovascular
 CALCIFICATIONS
 Small foci scattered throughout the tumor
 Interpreted as abortive enamel formation
 Larger areas of matrix material or calcification.
 Interpreted as dentinoid or cementum.
 Irregular round calcified bodies with concentric layered pattern may be
seen (Liesgang rings)
 OTHER
 Narrow, often anastomosing cords of epithelium are present in an

eosinophilic, loosely arranged matrix at the periphery of the tumor
adjacent to the capsule
 These cells are basaloid (resemble rests of dental lamina) and are
arranged in plexiform, cribriform, trabecular or lattice like configuration
(a) Lattice work pattern seen closer to the connective tissue capsule (H and E, ×10). (b) Dense hemmorhagic areas (H and E, ×10). (c) Irregular calcified bodies amidst the tumor cells (H a
×10). (d) Leisegang pattern of calcification in the form of globular or spheroidal masses (H and E, ×10)
SIGNIFICANCE
 The histopathologic features of this lesion are distinctive and should not be
confused with any other odontogenic tumor.
 May show focal areas that resemble CEOT, odontoma, or calcifying

odontogenic cyst.
 behave as a routine adenomatoid odontogenic tumor
 Unfamiliar pathologist may mistake this tumor for an ameloblastoma resulting in

unnecessary radical surgery.
 Because of its capsule, it enucleates easily from the bone.
 Aggressive behavior has not been documented, and
 Recurrence after enucleation seldom occurs

DENTINOGENIC GHOST CELL TUMOUR
CALCIFYING ODONTOGENIC CYST
Gorlin Cyst
Calcifying Cystic Odontogenic Tumor
Calcifying Ghost Cell Odontogenic Cyst
A rare, well circumscribed, solid or cystic lesion,

derived from odontogenic epithelium that
microscopically resembles ameloblastoma but
differs by containing ghost cells and spherical
tumours
CALCIFYING ODONTOGENIC CYST/ DENTINOGENIC
GHOST CELL TUMOUR
 Uncommon lesion with histopathologic diversity and variable clinical behavior
 Cystic or neoplastic lesion
 May be associated odontomas, adenomatoid odontogenic tumors and

ameloblastomas
 Revised who classification of odontogenic cysts & tumors have categorized COC
& DGCT into odontogenic cysts and tumours respectively
CALCIFYING ODONTOGENIC CYST/
CLINICAL FEATURES
 Both (Intra-/Extra osseous) forms occur with about equal frequency in the
maxilla and mandible.
 Predominantly in incisor and canine areas
 Wide age range with most cases diagnosed in the second and third decades of
life.
 Calcifying Odontogenic Cysts associated with Odontomas tend to occur in

younger patients, with a mean age of 17 years.
CLINICAL FEATURES
 Dentinogenic Ghost Cell Tumour appear to occur in older patients
 Extraosseous Calcifying Odontogenic Cysts are localized sessile or

pedunculated gingival masses with no distinctive clinical features
GHOST CELL TUMOUR
GHOST CELL TUMOUR
 Unilocular, well-defined radiolucency containing flecks of indistinct

radiopacities (salt pepper calcifications with equal diffuse distribution of RO &
RL)
 May occasionally appear multilocular.
 Irregular calcifications or toothlike densities, are present in about 1/3 rd-1/2 cases
 In 1/3rd cases, the radiolucent lesion is associated with an unerupted tooth (most
often a canine).
GHOST CELL TUMOUR
 2.0-4.0 cm in greatest D but lesions as large as 12.0 cm have been noted.
 Root resorption or divergence of adjacent teeth is seen with some frequency
 May closely resemble odontoma or ameloblastic fibro-odontoma in young

patients
 DGCT presents as an expansile radiopacity

GHOST CELL TUMOUR
GHOST CELL TUMOUR
GHOST CELL TUMOUR
GHOST CELL TUMOUR
GHOST CELL TUMOUR
Dentinogenic ghost cell tumour
 The cystic (nonneoplastic) forms comprise 86% to 98% of all

calcifying odontogenic cysts
 May occur both intraosseously and extraosseously.
 A well-defined cystic lesion

 Fibrous capsule
 Lining of odontogenic epithelium of 4 to 10 cells in thickness

 EPITHELIUM
 The basal cells of the epithelial lining may be palisaded cuboidal or

columnar similar to ameloblasts.
 The overlying layer of loosely arranged epithelium may resemble the

stellate reticulum of an ameloblastoma.
 EPITHELIUM
 Characteristic “ghost cells”
 Altered eosinophilic epithelial cells characterized by the loss of nuclei
with preservation of the basic cell outline
 Larger, vacuolated, contain remnants of nuclei and organelles (than
normal squamous cells)
 Origin is controversial
 Coagulative necrosis
 Accumulation of enamel proteins in the cells
 Abnormal keratinisation
 Epithelium may proliferate into lumen or capsule
GHOST CELL TUMOUR
 CONNECTIVE TISSUE
 Areas of an eosinophilic matrix material represent dysplastic dentin

(dentinoid) may be present adjacent to the epithelial component.
 the result of an inductive effect by the odontogenic epithelium on

the adjacent mesenchymal tissue
 When associated with odontoma, features of complex or compound

odontoma are seen
 CALCIFICATIONS
 Both in epithelium and CT

 Neoplastic (solid) COCs are uncommon, accounting for

2% to 16% of all calcifying odontogenic cysts
 May occur intraosseously or extraosseously (more

common).
HISTOPATHOLOGIC FEATURES (EXTRAOSSEOUS)
 EPITHELIUM
 Varying-sized islands of odontogenic epithelium in a fibrous
stroma.
 Peripheral palisaded columnar cells in epithelial islands
 Central stellate reticulum
 Nests of ghost cells within the epithelium
 Juxtaepithelial dentinoid
 D/D Peripheral ameloblastoma.

HISTOPATHOLOGIC FEATURES (INTRAOSSEOUS)
 Ameloblastoma-like strands and islands of odontogenic epithelium

in a mature fibrous connective tissue stroma.
 Variable numbers of ghost cells
 Juxtaepithelial dentinoid
GHOST CELL TUMOUR
 (a) Cystic lumen lined by prominent odontogenic epithelium with peripheral reactive bone. (b) Proliferating odontogenic epithelium forming follicles projecting into the fibrous connective tissue stroma.
(c) Areas of "ghost" epithelial cells projecting into the lumen (d) Ghost cells undergoing calcification
GHOST CELL TUMOUR
GHOST CELL TUMOUR
GHOST CELL TUMOUR
GHOST CELL TUMOUR
GHOST CELL TUMOUR
GHOST CELL TUMOUR
Odontogenic epithelium with dentinoid material (yellow arrow) and ghost cells (blue arrow)
ODONTOGENIC GHOST CELL CARCINOMA
(MALIGNANT VARIANT of DGCT)
 Cellular pleomorphism
 Mitotic activity with
 Invasion of the surrounding tissues.

Radiographic/Histo D/D
 CGCG
 Central Ossifying Fibroma
 AOT
 Ameoblastoma
 CEOT
 Ameloblastic fibro-odontoma
 Odontoma
COC
 Gorlin and his co-workers considered it to be ORAL

COUNTERPART of
 CUTANEOUS CALCIFYING EPITHELIOMA OF MALHERBE
(Pilomatricoma)
 Ghost cell or shadow cell keratinization is associated with epithelium of hair follicles
 Current immunological studies suggest that this lesion more

commonly resemble
 INTRACRANIAL CRANIOPHARYNGOMA
 Both lesions are positive for HMW-cytokeratins and Involucrin
CALCIFYING ODONTOGENIC CYST/ DENTINOGENIC GHOST CELL
TUMOUR
 The prognosis for a patient with a calcifying odontogenic cyst is good; only a few
recurrences after simple enucleation
 The peripheral neoplastic calcifying odontogenic cyst appears to have the same
prognosis as a peripheral ameloblastoma, with a minimal chance of recurrence after simple
surgical excision.
 When a calcifying odontogenic cyst is associated with some other recognized
odontogenic tumor, such as an ameloblastoma, the treatment and prognosis are likely to
be the same as for the associated tumor.
 Although few cases have been reported, odontogenic ghost cell carcinomas appear to
have an unpredictable behavior. Recurrences are common, and a few patients have died
from either uncontrolled local disease or metastases. An overall 5-year survival rate of
73% has been calculated for reported cases.
BENIGN ODONTOGENIC TUMOUR
5- SQUAMOUS ODONTOGENIC TUMOR
SQUAMOUS ODONTOGENIC TUMOR
A rare, sometimes multifocal potentially aggressive lesion derived from
odontogenic epithelium and consisting of islands of SSE that commonly
contains microcysts and calcifications in a dense fibrous CT
 Rare
 Mostly intraosseous
 Few peripheral
 Neoplastic transformation of dental lamina rests or
 The epithelial rests of malassez.
 The tumor appears to originate within the periodontal ligament
that is associated with the lateral root surface of an erupted tooth.
CLINICAL FEATURES
 Occur in patients with 8 to 74 years, peak in 3rd decade.
 They are equally distributed throughout the alveolar processes of the
maxilla and mandible, anterior to molar.
 no apparent sex predilection.
 A painless or mildly painful gingival swelling, often associated with
mobility of the associated teeth
 Small lesions appear as unilocular RL
 Large lesions as multilocular with indistinct margins
 consist of a triangular radiolucent defect lateral to the root or roots

of the teeth
 The radiolucent area may be somewhat ill defined or may show a

well-defined, sclerotic margin.
 <1.5 cm in greatest diameter.

 Varying-shaped islands of bland-appearing squamous epithelium in a mature
fibrous connective tissue stroma.
 The peripheral cells of the epithelial islands are cuboidal & do not show the
characteristic polarization seen in ameloblastomas
 The remaining cells are mature intermediate cells with prominent

desmosomal bridges
 Microcystic vacuolization and individual cell keratinization within the

epithelial islands are common features.
 Laminated calcified bodies and globular eosinophilic structures, which do

not stain for amyloid, are present within the epithelium or CT.
 D/D
 Similar islands of epithelium have been observed within the fibrous
walls of dentigerous and radicular cysts
 Conservative local excision or curettage appears to be effective for

patients with squamous odontogenic tumors, and most reported
cases have not recurred after local excision.

11-Odontogenic Tumours 2

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BENIGN EPITHELIAL

 “ A benign, locally aggressive tumor consisting of strands and

 Less than 1% of all odontogenic tumors.

 Central (Intraosseous) or Peripheral (Extra-osseous) forms

 Dental lamina remnants (anatomic distribution in the jaws)

 REE overlying the tooth crown

 The stratum intermedium of the enamel organ (resemblance to cells)

 Wide age range, most often between 30 and 50 years of

 2/3rd cases in the mandible, mostly posterior areas (molar >

 Clinically presents as a painless, slow growing swelling

 Nasal obstruction, epistaxis and proptosis in maxilla

 Peripheral (extraosseous) calcifying epithelial odontogenic tumor

 Unilocular or a multilocular radiolucent defect (unilocular pattern

 May be entirely radiolucent, but usually contains calciﬁed structures of

 Calciﬁcations are usually scattered within the tumor or are prominent

 Driven snow pattern is noted less frequently

 Well-defined scalloped margins,

 20% of cases have an ill-deﬁned periphery,

 Additional 20% exhibit a corticated border.

 Frequently associated with an impacted tooth, most often a mandibular

 Peripheral (extraosseous) calcifying epithelial odontogenic tumor is RL

 Cellular outlines of the epithelial cells are distinct, and intercellular

 Nuclear pleomorphism seen but not considered to indicate malignancy.

 Large areas of amorphous, eosinophilic, hyalinized (amyloid-

 The tumor islands frequently enclose masses of this hyaline

 Develop within the amyloid-like material

 Form concentric rings (liesegang ring calciﬁcations)

 These tend to fuse and form large, complex masses.

 The amyloid-like material in the pindborg tumor stains as

 APPLE-GREEN BIREFRINGENCE after congo red

 Some have large diffuse masses of amyloid-like material and

 lesions in the posterior maxilla should probably be treated more aggressively.

 A recurrence rate of about 15% has been reported;

 The overall prognosis appears good,

 Rare examples of malignant or borderline malignant calcifying epithelial odontogenic tumor

3. ADENOMATOID ODONTOGENIC TUMOR

usually occurs around the crown of un-erupted anterior teeth of young

patient and consists of epithelium in swirls and ductal patterns

interspersed with spherical calcifications

 3% to 7% of all odontogenic tumors

 Enamel organ epithelium,

 Remnants of dental lamina.

years of age.  Asymptomatic

 Striking tendency for ANTERIOR  Large lesions may cause painless

 Circumscribed, unilocular radiolucency involving the crown of an

 RL sometimes extends apically along the root past the CEJ

 Well-delineated unilocular radiolucency not related to an unerupted

 May be completely radiolucent; often contains ﬁne (snowﬂake)

 1. Spindle Shaped Epithelial Cells that form

 Whorled masses in a scant ﬁbrous stroma.

 This material may stain for amyloid.

 Narrow, often anastomosing cords of epithelium are present in an

 May show focal areas that resemble CEOT, odontoma, or calcifying

 Unfamiliar pathologist may mistake this tumor for an ameloblastoma resulting in

 Because of its capsule, it enucleates easily from the bone.

 Aggressive behavior has not been documented, and

 Recurrence after enucleation seldom occurs

A rare, well circumscribed, solid or cystic lesion,

 Uncommon lesion with histopathologic diversity and variable clinical behavior

 Cystic or neoplastic lesion

 May be associated odontomas, adenomatoid odontogenic tumors and

 Predominantly in incisor and canine areas

 Calcifying Odontogenic Cysts associated with Odontomas tend to occur in