Odontogenic tumors arise from tooth-forming tissues and can be divided into three categories: tumors of odontogenic epithelium without mesenchyme, tumors with both epithelium and mesenchyme, and tumors of mesenchyme alone. Ameloblastoma is the most common odontogenic tumor, representing 1% of jaw tumors. It typically presents as a multilocular radiolucency in the mandible and is classified as solid/multicystic, unicystic, or peripheral. Histologically it demonstrates islands of epithelial cells resembling dental lamina. Treatment involves wide local excision due to its persistence and recurrence.
2. INTRODUCTION
Group of lesions arising from the tooth- producing
apparatus or its remnants
May originate from epithelial and/or
ectomesenchymal odontogenic tissues
1% of all jaw tumors
2
3. WHO HISTOLOGICAL TYPING OF ODONTOGENIC
TUMORS
1992 (MODIFIED)
Tumors of odontogenic epithelium without
odontogenic ecto-mesenchyme
Tumors of odontogenic epithelium with odontogenic
ecto-mesenchyme with or without dental hard tissue
formation (mixed)
Tumors of odontogenic ecto-mesenchyme with or
without odontogenic epithelium
3
5. TUMORS OF ODONTOGENIC EPITHELIUM WITH
ODONTOGENIC ECTO-MESENCHYME WITH OR
WITHOUT DENTAL HARD TISSUE FORMATION (MIXED)
• Ameloblastic fibro odontoma
• Adenomatoid odontogenic tumor (AOT)
• Ameloblastic fibroma
• Odontomes
Compound
Complex
5
6. TUMORS OF ODONTOGENIC ECTO-MESENCHYME WITH
OR WITHOUT ODONTOGENIC EPITHELIUM
Odontogenic fibroma
Odontogenic myxoma
Cementoblastoma
6
7. MALIGNANT ODONTOGENIC TUMORS
Odontogenic carcinoma
a. Malignant ameloblastoma
b. Primary intraosseous carcinoma
c. Malignant variant of other odontogenic epithelial tumors
d. Malignant changes in odontogenic cyst
Odontogenic sarcoma
a. Ameloblastic fibrosarcoma
b. Ameloblastic fibrodentinosarcoma
7
10. TUMORS OF ODONTOGENIC EPITHELIUM WITH ODONTOGENIC
ECTO-MESENCHYME WITH OR WITHOUT DENTAL HARD TISSUE
FORMATION
• Ameloblastic fibroma
• Ameloblastic fibrodentinoma (dentinoma)
• Ameloblastic fibro odontoma
• Odontomes
Compound type
Complex type
Odontoameloblastoma
Calcifying cystic odontogenic cyst
Dentinogenic ghost cell tumor
10
11. TUMORS OF MESENCHYME AND/ OR ODONTOGENIC ECTO-
MESENCHYME WITH OR WITHOUT ODONTOGENIC EPITHELIUM
Odontogenic fibroma
Odontogenic myxoma/ Myxofibroma
Cementoblastoma
11
12. BONE RELATED LESIONS
Other tumors – Melanotic neuroectodermal tumor of infancy
12
• Ossifying fibroma (Cemento Ossifying fibroma)
• Fibrous dysplasia
• Osseous dysplasia
• Cemento osseous dysplasia
Periapical cemental dysplasia
Focal cemento osseous dysplasia
Florid cemento osseous dysplasia
• Central Giant cell granuloma
• Cherubism
• Aneurysmal bone cyst
• Simple bone cyst
13. MALIGNANT TUMORS OF ODONTOGENIC ORIGIN
Odontogenic carcinoma
a. Malignant ameloblastoma
b. Ameloblastic carcinoma: Primary type (intraosseous)
c. Ameloblastic carcinoma: Secondary type (peripheral)
d. Primary intraosseous squmaous cell carcinoma
e. Primary intraosseous squmaous cell carcinoma derived
Keratocytsic odontogenic tumor
f. Primary intraosseous squmaous cell carcinoma derived from an
odontogenic cyst
g. Clear cell odontogenic carcinoma
h. Ghost cell odontogenic carcinoma
Odontogenic sarcomas
a. Ameloblastic fibrosarcoma (ameloblastic sarcoma)
b. Ameloblastic fibrodentinoma sarcoma & fibro odontosarcoma
13
14. 14
2017 WHO classification of
odontogenic tumors and cysts
Malignant odontogenic
tumors
Benign odontogenic
tumors
• Epithelial origin
• Mixed (Epi-mes)
origin
• Mesenchymal origin
22. DEFINITION
Ameloblastoma is a tumor that is “usually
unicentric, non-functional, intermittent
in growth, anatomically benign and
clinically persistent”.
-Robinson
22
23. HISTOGENESIS
Cell rests of the enamel organ
(remnants of dental lamina or HERS)
Epithelium of odontogenic cysts (esp. D C)
Disturbance of the developing enamel organ
Basal cells of oral mucosal epithelium
Heterotopic epithelium
23
33. Mixture of different patterns is
common
A lesion may exhibit one or more
histologic patterns
Lesions are sub-classified according
to the most predominant pattern
33
34. FOLLICULAR AMELOBLASTOMA
Islands of epithelial cells in a connective
tissue stroma
Epithelial islands have a core of loosely
arranged polygonal cells (resembling stellate
reticulum), surrounded by a peripheral layer
of tall, columnar ameloblast-like cells with
polarized nuclei
Cyst formation: microcysts to generally cystic
tumour (degeneration of stellate reticulum-like
cells).
34
45. GRANULAR CELL AMELOBLASTOMA
Relatively rare subtype
Resembles follicular type, but center of the
tumour islands form sheets of large
eosinophilic granular cells
Cytoplasmic granules consist of pleomorphic,
lysosome-like organelles
Aggressive variant with highest recurrence
rate
45
52. BASAL CELL AMELOBLASTOMA
Least common type
Occurs primarily in extra-osseous lesions
Nests of basaloid cells occupy the central
portions of the islands (no stellate reticulum-like
tissue)
52
56. PLEXIFORM AMELOBLASTOMA
Network of thin (inter-connecting)
strands of epithelium bordered by
columnar cells
Loose, vascular, sparsely cellular or
fibrous stroma may show areas of
cystic degeneration
56
70. Intraluminal / Plexiform unicystic
ameloblastoma:
Intraluminal plexiform proliferation, but no
infiltration of the cyst wall
(i.e., projects only into the lumen).
70
72. Mural ameloblastoma
Invasive islands of follicular or plexiform type of
ameloblastoma, sometimes continuous with cyst
lining, infiltrate the cyst wall.
72
80. RADIOGRAPHIC FEATURES
Rarely produces saucerization of underlying
bone
HISTOLOGIC FEATURES
Mostly acanthomatous > plexiform >
follicular type
80
84. Craniopharyngioma/Pituitary ameloblastoma
(copious ghost cell production, cystic morphology, and
the presence of osteoid or bone).
Adamantinoma of long bones
(lack the regular shape and reversed nuclear polarity of
ameloblastoma cells).
84
Extra-oral tumours resembling
Ameloblastoma
85. MALIGNANT AMELOBLASTOMA
An ameloblastoma that has given rise
to pulmonary or nodal metastases and
the metastases have retained the
microscopic appearances of the
primary growth
85
86. AMELOBLASTIC CARCINOMA
Ameloblastoma that has cytologic features
of malignancy in the primary lesion or in a
metastasis. Later, the metastases tend to
resemble less well-differentiated squamous
cell carcinoma
86
89. HISTOGENESIS
From stratum intermedium (cell resemble &
high alk phosphatase activity)
Reduced enamel epithelium?
89
90. CLINICAL FEATURES
Occurs over a wide range from 3-92
years; (average = 40 years)
Mandible > maxilla; molar region
Painless, slow-growing swelling
May be associated with unerupted or
impacted tooth (third molars)
90
92. RADIOGRAPHIC FEATURES
May exhibit considerable variation
• Diffuse or well-circumscribed radiolucent area
(may be with an impacted tooth)
• Mixed radiolucent / radio-opaque picture with
many small irregular bony trabeculae traversing
the radiolucent area
• Multilocular or ‘honeycomb’ pattern
• Scattered flecks of calcification in the RL areas-
‘driven –snow’ appearance
• Peripheral CEOT- saucerization of underlying
92
94. HISTOLOGIC FEATURES
Islands, sheets or strands of pleomorphic, slightly
eosinophilic epithelial cells in a connective tissue
stroma
Distinct cell membranes and intercellular bridges
of epithelial cells
Nuclei vary in number, size and shape
Mitosis is rare
Presence of a homogeneous eosinophilic
amyloid-like substance, which may calcify to form
concentrically lamellated ‘Liesegang rings’
94
96. TUMOR CELLS WITH LARGE CENTRALLY LOCATED
HYPERCHROMATIC NUCLEI
96
97. TUMOR CELLS WITH VARIABILITY IN NUCLEAR SIZE, SHAPE AND
STAINING
97
98. 98
Amyloid- homogenous eosinophilic substance
Thought to be
• Filamentous degeneration of keratin
filaments conversion into amyloid
• Tissue degeneration
• Type IV collagen
• Basal lamina
• Enamel matrix
101. CALCIFICATION OF AMYLOID-LIKE DEPOSITS
101
• Presence or absence of calcification in
CEOT has prognostic implications
• Lack of calcification indicates less tumor
differentiation and hence favors more
chance of recurrence
102. CONCENTRIC LAYERS OF CALCIFICATION WITHIN AMYLOID-LIKE
MATERIAL LIESEGANG RINGS
102
104. CLEAR CELL VARIANT OF CEOT
Cells have clear vacuolated cytoplasm with
round or oval nuclei, or nuclei flattened
against the cell membrane
Clear cells comprise the bulk of the tumour,
or are seen in a few scattered foci
Clear cells are mucicarmine-negative
104
109. ADENOMATOID ODONTOGENIC
TUMOUR
(AOT)
(Adenoameloblastoma,
Ameloblastic adenomatoid tumour)
Characterised by formation of duct-like structures by
epithelial component of the lesion
Uncommon; completely benign; may be hamartomas
1992 WHO classification it was under epi-mes tumor
2005 WHO classification & 2017 its under epithelial
without ectomesenchyme origin
109
111. CLINICAL FEATURES
Slow growing, painless swelling;
< 3cm in size
May be associated with an
unerupted tooth (cuspid)
111
112. 112
2/3rd of cases occur in 2nd decade
2/3rd of cases occur in females
2/3rd of cases occur in ant maxilla
2/3rd of cases associated with
unerupted canine
2/3rd tumor
113. RADIOGRAPHIC FEATURES
Rounded radiolucency with well-
defined outline with faint radio-opaque
foci
(‘snow-flake’ calcifications) in 60% of
cases.
Displacement of adjacent teeth / roots;
resorption is rare
113
114. 114
2 variants :
1. Follicular variant – tumour contains a tooth
2. Extra follicular variant – no tooth in the
tumour; seen between roots of teeth
116. HISTOLOGIC FEATURES
Well-defined capsule
Spindle-shaped or cuboidal epithelial
cells in sheets, whorls, strands; may
form rosettes
Columnar or cuboidal cells arranged in
ductal pattern, with ductal lumina
containing eosinophilic coagulum
116
117. HISTOLOGIC FEATURES
Loose, scanty, sparsely cellular connective
tissue stroma
Fragments of amorphous or crystalline
calcification (possibly amyloid) seen among
sheets of epithelial cells
Small foci of calcification scattered throughout
the tumour (attempted formation of enamel or
dentine or cementum)
117
128. Complete differentiation of epithelial
and mesenchymal cells
→ functional ameloblasts and odontoblasts
→ enamel and dentin formation
Improper morpho-differentiation of
odontogenic cells
→ haphazard deposition of enamel and dentin
128
133. CLINICAL FEATURES
Any age
any site
maxilla : mandible: : 2:1
right side > left
compound > complex
compound – anterior maxilla
complex – posterior jaws
133
134. Small
Asymptomatic, but may result in unerupted /
impacted teeth, retained deciduous teeth
134
135. RADIOGRAPHIC FEATURES
Irregular mass of calcified material / variable
number of tooth like structures surrounded by
a narrow radiolucent band with a smooth
periphery
Between the roots of teeth
Associated with unerupted /impacted tooth
135
138. HISTOPATHOLOGICAL FEATURES
Normal appearing dental tissues
(that may or may not resemble
normal teeth) in a fibrous connective
tissue
Presence of ‘ghost’ cells
138
150. Neoplasms composed of proliferating
odontogenic epithelium embedded in a
cellular ectomesenchymal tissue that
resembles the dental papilla, with varying
degrees of inductive change and dental
hard tissue formation.
(WHO definition, 1992)
150
151. Simultaneous proliferation of both
epithelial and mesenchymal tissue
without formation of enamel or
dentin
151
152. CLINICAL FEATURES
posterior region of mandible
14.6 yrs
♂
mostly asymptomatic; sometimes
associated with pain, tenderness or
mild swelling
152
169. INDUCTION OF THIN LAYER OF
ATUBULAR DENTIN IN THE STROMA BY
THE AMELOBLAST-LIKE CELLS
169
170. PRIMORDIAL ODONTOGENIC TUMOR
1st described by Mosqueda-Taylor et al
Benign odontogenic tumor developing during
primordial stages of tooth developemnt
Classified under mixed odontogenic tumors- dental
papilla surrounded by delicate ameloblastic
epithelium
Only around 14 cases reported until now
Occurs in young age- 2nd decade of life
170
171. PRIMORDIAL ODONTOGENIC TUMOR
Slight male predilection
Mandible the most common site; unerupted tooth
Asymptomatic swelling presenting with expansion
of cortical bone
171
172. X- ray:
large well defined radiolucency
involving an completely unerupted tooth – 3rd
molar
Root resorption seen
Buccal and lingual cortical plate expansion
Gross findings
Encapsulated solid nodule, pale, slippery
172
175. Histopathology
Variably cellular to loose fibrous tissue with
areas mimicking dental papilla surrounded
by epithelial cells – cuboidal to columnar
No hard tissue formation
Calcifications may be seen in myxoid CT
stroma
175
177. Treatment
Enucleation and extraction of involved tooth
Conservative surgical excision
Prognois – good
Recurrences- rare
177
178. DENTINOGENIC GHOST
CELL TUMOR
COC was first described by Gorlin and his colleagues in 1962,
Controversy as to whether COC is a cyst or a tumor
Two organizing principles of classification of
Monistic concept: postulates that all COCs are neoplastic in
nature, even though the majority are cystic in architecture and
appear to be non-neoplastic
Dualistic concept favored by most researchers proposes that
COCs contain two different entities- cyst & Tumor
Cyst- “Calcifying Cystic Odontogenic Tumors” (CCOT)
Neoplasm-“Dentinogenic Ghost Cell Tumor” (DGCT)
178
179. DGCT term 1st proposed by Praetorius et al. in 1981 for
the neoplastic variety of COC, i.e., the Type 2 of COC
DGCT has also been termed as odontogenic ghost cell
tumor by Colmenero et al.
DGCT is an extremely rare odontogenic tumor
Central and a peripheral type
179
Histogenesis- cell rests of Serre or the surface epithelium but
unclear
β-catenin plays an important role in the tumorigenesis of DGCT
by an improper differentiation process coordinated by Wnt
signaling pathway
180. Age- 50yrs (17-72yrs)
Slight male predilection
Maxilla = Mandible
Canine to 1st molar region affected
Asymptomatic – few pain & discomfort
Tooth bearing area or edentulous jaw
180
181. X rays shows
Unilocular & multilocular
Radiolucency with scattered radio-opaque calcifications
Occlusal radiographs show a bicortical expansion
Root resorption or an impacted tooth in relation to the tumor
mass is also noted in some cases
181
183. Histopathology
Islands of odontogenic epithelium
Tall columnar ameloblast-like cells with hyperchromatic nuclei
Large areas of eosinophilic globules suggestive of dentinoid
Ghost cells showing keratinization and calcification
183
Dentin like
Calcification in
ghost cells
Giant cell
184. Treatment
Enucleation or surgical resection- enbloc, segmental
Locally aggressive lesion
Recurrence rate is high (71%)
Malignant transformation has been reported
184
186. Small focal excessive mass of enamel
(ectopic nodule) on the surface of a
tooth root
186
187. At furcation of roots or near the CEJ
Maxillary molars > mandibular molars
Asymptomatic
Higher incidence in Mongoloid and
Eskimo populations
187
188. ETIOPATHOGENESIS
localised bulging of the odontoblastic layer
↓
prolonged contact between HERS and the
developing dentin
↓
inductive influence
↓
formation of enamel
188
191. May consist purely of
enamel; may also contain
underlying dentin and
pulp tissue
191
192. Potential complications:
Weak periodontal attachment; attachment
by hemidesmosomes
Area of stagnation that favours plaque
retention and improper cleansing
Enamel pearl may sometimes contain vital
pulp tissue
192
201. THREE BASIC CONCEPTS REGARDING
THE TUMOUR :
Lesion around the crown of an unerupted
tooth resembling a dentigerous cyst
Lesion of fibrous connective tissue with
scattered islands of odontogenic epithelium
Fibroblastic neoplasm containing varying
amounts of odontogenic epithelium and
calcified material resembling dysplastic
dentin / cementum
201
202. CLINICAL FEATURES
Age: 1st – 8th decades (mean age: 40 years)
Female >males
Mandible > maxilla
Maxilla – (lesions located anterior to I molar)
Mandible – (lesions located posterior to I molar)
Asymptomatic
May be associated with an unerupted tooth
202
203. RADIOGRAPHIC FEATURES
Smaller lesions:
well-defined unilocular radiolucencies
larger lesions → multilocular
Lesions are seen in the peri-radicular
area; may cause root resorption
203
206. SIMPLE COF:
Tumour mass composed of mature collagen
fibres with stellate fibroblasts in a whorled
pattern
Small islands / nests of inactive odontogenic
epithelium
206
213. CLINICAL FEATURES
2nd and 3rd decade of life;
(rare before age of 10 years or after 50)
Males = Females
Mandible > Maxilla
Usually asymptomatic
Rare lesions expand the bone and may
cause destruction of cortex
213
214. RADIOGRAPHIC FEATURES
Unilocular / multilocular radiolucent lesion
May have a mottled appearance
May cause displacement / resorption of teeth
The radiolucent area may contain thin wispy
trabeculae of residual bone arranged at right
angles to one another
‘Tennis-racket’ / ‘step-ladder’ appearance
214
218. HISTOLOGICAL FEATURES
Loosely arranged spindle-shaped and
stellate cells with long fibrillar processes
Myxoid intercellular substance with few
collagen fibrils
The ground substance contains two acid
mucopolysachharides
(↑ hyaluronic acid; ↓ chondroitin sulfate)
218
222. Tumour cells exhibit high alkaline
phosphatase and lactate dehydrogenase
activity
Ultrastructural studies show two types of
cells
Pale cells (represent secretory cells; synthesis of
mucopolysaccharide)
Dark cells (contain crystallised collagen fibrils)
222