2019

Nguyen Thi Hong, D.D.S., Ph.D.

Hong Bang International University
 Learning Objectives

1. Define classification and terminology of OT

2. Define epidemiology of important OT
3. Apply key diagnostic features of common OT to
diagnose OT cases
4. Identify clinicopathological features which may
indicate malignancies of OT
5. Choose effective treatment methods for OT
 Structure

1. Classification and epidemiology

2. Benign odontogenic tumors
3. Malignant odontogenic tumors
CLASSIFICATION
&
EPIDEMIOLOGY
 Odontogenic tumors - classification

BENIGN
• Odontogenic epithelium only
• Odontogenic epithelium & odontogenic mesenchyme
• Odontogenic mesenchyme only

MALIGNANT
• Carcinomas & Sarcomas
Odontogenic Tumors – WHO classification, 2017

Benign epithelial odontogenic tumors

1. Ameloblastoma (u nguyên bào men)
Ameloblastoma, unicystic type (UNBM đơn nang)
Ameloblastoma, extraosseous/peripheral type
(UNBM ngoài xương/ngoại vi )
Metastasizing ameloblastoma (UNBM di căn)
2. Squamous odontogenic tumor (u gai do răng)
3. Calcifying epithelial odontogenic tumor (u biểu mô do răng
canxi hóa)
4. Adenomatoid odontogenic tumor (u dạng tuyến do răng)
Benign mesenchymal odontogenic tumors
5. Odontogenic fibroma (u sợi do răng)
6.Odontogenic myxoma/myxofibroma (u nhầy do răng)
7.Cementoblastoma (u nguyên bào xê-măng)
8.Cemento-ossifying fibroma (u sợi hóa xê-măng)

Benign mixed epithelial & mesenchymal odontogenic tumors

9. Ameloblastic fibroma (u sợi nguyên bào men)
10. Odontoma (u răng)
11. Dentinogenic ghost cell tumor (u tế bào bóng ma do răng)
12. Primordial odontogenic tumor (u nguyên thủy do răng)
Odontogenic Tumors – WHO classification, 2017

Malignant odontogenic tumors

13. Odontogenic carcinomas (carcinoma do răng)
14. Ameloblastic carcinoma (carcinoma nguyên bào men)
15. Primary intraosseous carcinoma, NOS (carcinoma nguyên
phát trong xương, không đặc hiệu)
16. Sclerosing odontogenic carcinoma (carcinoma xơ hóa do
răng)
17. Clear cell odontogenic carcinoma (carcinoma tế bào sáng
do răng)
18. Ghost cell odontogenic carcinoma (carcinoma tế bào bóng
ma do răng)
19. Odontogenic carcinosarcoma (carcinoma-sarcoma do răng)
20. Odontogenic sarcomas (sarcoma do răng)
 Odontogenic Tumors
- Epidemiology -

• Rare (< 1% of oral tumors)

• Most common tumors in jaws
• Odontoma is most common lesion
• Ameloblastoma is most common neoplasm
Lesions of the jaws:

• Odontogenic cysts 90%

• Odontogenic tumors 5%
• Fibro-osseous lesions 4%
• Bone tumors 1%
 Jaw Lesions
100

90
79
80
Children
70
Adults
60 55

% 50

40

30 26

20 15
13
10 4 4 3
0
Odont cysts Odont Tum Bone lesion Non-Odont
cysts

Jones & Franklin. Int J Paed Dent. 2006. n = 941

National Hospital of Odonto-Stomatology, HCMC city (NHOSH) - 2007 & 2008

Tumors of the jaws Cases %

Ameloblastoma 52 30
Odontoma 20 12
CEOT 5 3
Cementoblastoma 2 1
Fibrous dysplasia 48 28
Osteoma 25 15
Myxoma 4 2
Chondroma 4 2
Plasmacytoma 3 2
Giant cell tumor 1 1
Lipofibroma 1 1
Malignant tumors 4 2
 Odontogenic Tumors
- Etiology & Pathogenesis -

• Etiology is unknown.
• Many genetic alterations have been described.
• Closely related to mechanisms of tooth development.
 Early stages of Odontogenesis

1. Invagination
2. Cap stage Sapp et al., 2004

3. Early bell stage

4. Late bell stage
 Late stages of Odontogenesis

5 6 7 Sapp et al., 2004

5. Crown formation & remnants of dental lamina

6. Root formation
7. Preeruption completed tooth formation & rests of
Malassez
 ODONTOGENIC TUMORS

Pathogenesis: 3 steps of tooth germ formation:

1. Proliferation
2. Differentiation
3. Organization

Disorder

Odontogenic tumors
 BENIGN
ODONTOGENIC TUMORS
1. Benign epithelial odontogenic tumors
2. Benign mesenchymal odontogenic tumors
3. Benign mixed epithelial & mesenchymal
odontogenic tumors
 Benign
Epithelial Odontogenic tumors

1. Ameloblastomas
2. Adenomatoid Odontogenic Tumor (AOT)
3. Calcifying Epithelial Odontogenic Tumor (CEOT)
4. Squamous Odontogenic Tumor (SOT)
AMELOBLASTOMA
 AMELOBLASTOMA

 Terminology:
Adamantinoma (1885, Malassez)
Ameloblastoma (1934, Churchill)

 Definition:
A locally invasive neoplasm of odontogenic epithelium
that has a wide spectrum of histopathologic patterns
resembling early odontogenesis.
(Sapp, 2002)
Odontogenic epithelium sources of Ameloblastoma

Sapp et al., 2004

1. Remnants of dental lamina (Rests of Serres)

2. Enamel epithelium
3. Rests of Malassez
4. Basal cell layer of overlying surface epithelium
 AMELOBLASTOMA

The most representative odontogenic tumor:

50% of ODTs in Asia
10-20% in Western countries

4 Subtypes of Ameloblastoma (WHO 2017)

1. Conventional Ameloblastoma (Solid / Multicystic)
2. Unicystic Ameloblastoma
3. Peripheral Ameloblastoma
4. Metastasizing Ameloblastoma
 AMELOBLASTOMA
- clinicopathological subtypes -

Sapp et al., 2004

Solid / Multicystic Unicystic Peripheral

or Conventional or Extraosseous

Very important to know which as not all require same treatment

 Conventional Ameloblastoma
(Solid / Multicystic Ameloblastoma) (SMA)
Solid / Multicystic Ameloblastoma (SMA)
- clinical features -

 80-85% of ameloblastomas
 Age: 20-40 or 30-50
 80% in mandible
(most at angle of mandible)
 Slow-growing but locally
aggressive
 Intraosseous location but cortical
bone perforation may occur
 High rate of recurrence if not
Regezi et al, 2016
removed adequately
 Solid / Multicystic Ameloblastoma (SMA)
- radiological features -
 Multilocular radiolucency >> Unilocular radiolucency
 “soap bubble” or “honeycombed”
 Often a scalloped margin
 Cortical expansion
 Clear-cut root resorption
 40% associated with impacted teeth
Solid / Multicystic Ameloblastoma (SMA)
- histopathology -

Sapp et al., 2004

Solid / Multicystic Ameloblastoma (SMA)
- histopathology -

Follicular pattern Plexiform pattern

 Solid / Multicystic Ameloblastoma (SMA)
6 histopathologic subtypes

1. Follicular pattern
2. Plexiform pattern
3. Acanthomatous pattern
4. Granular cell pattern
5. Basal cell pattern
6. Desmoplastic pattern

Similar biologic behavior → Treatment is same for all

 Solid / Multicystic Ameloblastoma (SMA)
- treatment & prognosis -

 DO NOT: Enucleate – recurrence high (90%)

 Bloc resection (recurrence 15%)
 Prognosis good (benign tumor);
Site: mandibular better > maxilla
 Malignant transformation (1%):
- Malignant ameloblastoma
- Ameloblastic carcinoma
Unicystic Ameloblastoma
 Unicystic Ameloblastoma (UA)
- clinical & radiographical features -
 Present clinically and histologically as a cyst
 Younger age (16-20 years), Male = Female
 90% in mandible (esp. posterior)
 80% associated with unerupted lower 3rd molars
 Usually unilocular > multilocular radiolucency
 Not infiltrative, enucleate easily and good prognosis

Gardner DG et al
Head and Neck tumors, WHO, 2005
Unicystic Ameloblastoma (UA)
- histopathology -

1. Simple UA
2. Luminal (plexiform) UA
3a, 3b. Mural UA
 Unicystic Ameloblastoma (UA)
- treatment & prognosis -

– Enucleation / Simple UA, Luminal UA

– Resection / Mural UA
– Recurrence 10 – 20%; good prognosis

Regezi et al, 2016

Ameloblastoma

Cawson RA & Odell EW

Cawson‟s Essentials of Oral Pathology and Oral
Medicine, Elsevier, 2002
 Peripheral Ameloblastoma (PA)
(Extraosseous Ameloblastoma)
 1 – 10% of ameloblastomas
 Epulis-like tumor, sessile, ± erythematous & ulcer, firm
 70% in lower gingiva, esp. in anterior to premolar area.
 Male:Female ratio = 2:1; average age = 52
 Histology is same as central ameloblastoma
 Treatment: excision; low recurrence rate

E-PIE, 2004
 Metastasizing Ameloblastoma (MA)

 Average age: 30 - 40
 Histologically benign appearance of
ameloblastoma but metastasize to lymph node
and/or distant organs (lung)
 Treatment: block resection
 Prognosis: moderate – 50%

Lymph node metastasis

of ameloblastoma
ADENOMATOID ODONTOGENIC TUMOR (AOT)
(Ameloblastic Adenomatoid Tumor)
(Adeno-ameloblastoma)

 Pathogenesis: derived from odontogenic epithelium

(ameloblasts, Serres)
 Clinical featrues:
• 3 - 7%
• Age: 15 - 20, Female > Male
• Maxilla > Mandible (2:1), esp. cuspid.
• Swelling over an unerupted tooth.
 Adenomatoid Odontogenic tumor (AOT)

 Radiographic features: unilocular radiolucency,

well-cortinated borders, ± flecks of radiopacities,
containing a tooth (75%)
 Histopathology: capsule, epithelium in swirls &
ductal pattern, ± calcification.
 Treatment:
enucleation +
removal of
associated teeth.
Sapp et al., 2004
SQUAMOUS ODONTOGENIC TUMOR (SOT)
 Rare
 Pathogenesis: derived from the remnants of dental
lamina, rests of Malassez or overlyinng epithelium
 Clinical features: either jaws, anterior to the molars,
age 20-70 (mean age: 40 years)
 Radiographic features: uni-/multilocular radiolucency,
indistinct border
 Histopathology: islands of normal stratified squamous
epithelium in the connective tissue stroma
 Treatment: curettage / small lesion
block resection / large lesion
CALCIFYING EPITHELIAL ODONTOGENIC TUMOR (CEOT)
(Pindborg Tumor)

 Rare
 Similar to solid ameloblastoma: age, site, local invasion
 Accompanied by spherical calcification & amyloid
 Treatment: block resection

Sapp et al., 2004

Benign Mesenchymal
Odontogenic Tumors
1. Odontogenic Fibroma
2. Odontogenic Myxoma
3. Cementoblastoma
 ODONTOGENIC FIBROMA

 Very rare
 Pathogenesis: dental follicle or periodontal ligament?
 Clinical features: wide age range (10-70), most
common in posterior mandible
 Radiological features: Uni/Multilocular cystic
radiolucency, well-circumscribed margin.
 Histopathology: odontogenic epithelium, ± calcified
material- osteoid or „dentinoid‟
 Treatment: Enucleation
 ODONTOGENIC MYXOMA

 Benign but locally invasive

 Pathogenesis: not clear
• No evidence for odontogenic origin
• BUT - almost specific to jaws.
 Clinical features:
• Wide age range (esp. 20-30 yrs)
• 66% in mandible (usually in molar region)
• Painless swelling.
 Odontogenic Myxoma

 Radiographic features: # solid ameloblastoma BUT

- root resorption is not a feature of myxoma
 Histopathology: no capsule, spindled cells in a
mucoid stroma
 Treatment: block resection
Vimentin +
SMA +
S100 +/-
 CEMENTOBLASTOMA

 A benign, well-cirumscribed neoplasm of cementum

-like tissue growing in continuity with the apical
cemental layer of a molar or premolar.
 Clinical features:
• Age 10-40 (peak 19)
• Usually mandible (75%)
• Molar teeth (50%)
• Swelling, painful (50%)

 Radiopaque lesion attached to tooth root (vital tooth)

 Capsule -> Treatment: Enucleation + tooth extraction.
Mixed Odontogenic tumors

1. Ameloblastic fibroma (AF)

2. Ameloblastic fibro-odontoma (AFO)
3. Odontoma
4. Dentinogenic ghost cell tumor
5. Primordial odontogenic tumor
 AMELOBLASTIC FIBROMA (AF)

 Age: 10-20
 Swelling, over unerupted molars in the mandible
 Unilocular or Multilolcular radiolucency
 Circumscribed → Treatment: careful enucleation

Regezi et al, 2016 Sapp et al, 2004

AMELOBLASTIC FIBRO-ODONTOMA (AFO)

 Age: 10 - 20
 Capsule lesion, containing ameloblastic fibroma &
complex odontoma.
 Mostly in the posterior areas of the mandible
 Unilocular, well-circumscribed mixed radiolucent &
radiopaque lesion, ± containing impacted tooth.
 Treatment is same as ameloblastic firboma (careful
enucleation).
DENTINOGENIC GHOST CELL TUMOR
 ODONTOMA

 ~ 70% of all odontogenic tumors.

 Hamartomatous lesion
 Contaning enamel, dentin, pulp, cementum in
tooth shapes (compound) or a solid gnarled mass
(complex)
 Age 10-20
 Male ~ Female
Compound odontoma:
 Maxilla > Mandible
Anterior region
 Containing small
tooth like structures

Sapp et al, 2016

Complex odontoma:
Mandible > Maxilla
Especilally in
premolar/molar region
Regezi et al, 2016
 MALIGNANT
ODONTOGENIC TUMORS

1. Odontogenic carcinomas
2. Odontogenic sarcomas
Very rare
 ODONTOGENIC CARCINOMAS

1. Ameloblastic carcinoma
2. Primary intraosseous squamous cell carcinoma
3. Clear cell odontogenic carcinoma
4. Ghost cell odontogenic carcinoma
 Ameloblastic Carcinomas (AC)

 Microscopic pattern of ameloblastoma but with

cytologic signs of malignancy.
 Treatment: Radical resection + Radiation
 Prognosis: very poor (5yr survival – 30%)
 Primary Intraosseous
Squamous Cell Carcinoma (PISCC)

• de novo from odontogenic epithelial remnants or

derived from odontogenic cysts
• Age: 40-70
• Usually in the body or posterior mandible
• Irregular radiolucency
• Composed of sheets of squamous cell carcinoma

Eversole et al
Head and Neck tumors, WHO, 2005
 Clear Cell Odontogenic Carcinoma

• Low-grade malignancy
• Elderly patients, mandible or maxilla
• Radiolucency with poorly defined margins
• 10% nodal metastasis
• Composed of sheets of infiltrating clear cells

Cawson RA & Odell EW, 2002

Ghost Cell Odontogenic Carcinoma (GCOC)

 Age: 4th decade,

 Male: Female ratio = 2:1; Maxilla > Mandible
 Osteolytic radiolucency, some radiopaque material
 Malignant epithelial cells with ghost cells
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