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Odontogenic Tumors

Odontogenic Tumors. Assoc. Professor Jan Laco, MD, PhD. Causes of jaw swelling. Odontogenic cysts Odontogenic tumors Giant cell lesions Fibro-osseous lesions Primary (non-odontogenic) tumors of bone Metastatic neoplasms Chronic osteomyelitis. Odontogenic tumors.

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Odontogenic Tumors

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  1. Odontogenic Tumors Assoc. Professor Jan Laco, MD, PhD

  2. Causes of jaw swelling • Odontogenic cysts • Odontogenic tumors • Giant cell lesions • Fibro-osseous lesions • Primary (non-odontogenic) tumors of bone • Metastatic neoplasms • Chronic osteomyelitis

  3. Odontogenic tumors • derived from epithelial, ectomesenchymal, mesenchymal elements of tooth forming apparatus • central (intraosseous) • maxillofacial skeleton • peripheral (extraosseous) • soft tissue - gingiva + alveolar mucosa • ethiology unknown, from some odontogenic cysts • RTG • uni-, multi-locular radiolucencies ~ cysts x radiopacities • !!! biopsy !!!

  4. 1. Epithelial ameloblastoma squamous odontogenic tumor calcifying epithelial odontogenic tumor adenomatoid odontogenic tumor 2. Epithelial+mesenchymal ameloblastic fibroma odontoma dentinogenic ghost cell tumor primordial odontogenic tumor Odontogenic tumors

  5. 3. Mesenchymal odontogenic fibroma odontogenic myxoma cementoblastoma 4. Carcinomas ameloblastic carcinoma primary intraosseous squamous cell carcinoma clear cell odontogenic carcinoma ghost cell odontogenic carcinoma sclerosing odontogenic carcinoma 5. Odontogenic carcinosarcoma 6. Odontogenic sarcoma Odontogenic tumors

  6. Ameloblastoma • benign x locally aggressive • 1. solid / multicystic A (S/MA) • 2nd most common odontogenic tumor • M ~ F, peak 30-50Y; RTG: multilocular cystic radiolucency • posterior mandible (70%) +  posterior maxilla ( skull) • spread through bone medullary spaces, cortex intact • Mi: follicular / plexiform pattern + fibrous stroma • islands of odontogenic epithelium (enamel organ-like) • basaloid, granular, acanthomatous variants, keratoA • anastomosing strands • recurrence (after 10Y) – long term RTG follow-up !!! • treatment: jaw resection with free margins (2 cm)

  7. Ameloblastoma • 2. extra-osseous (peripheral) A • ~ S/MA x soft tissues over mandible, treat.: simpleexcision • older pts., M : F …1 : 2 • 3. unicystic A • 2 peaks – 16Y (+ uneruptedtooth) + 35Y (NO uner. tooth) • 5-15% of all As, luminal x muralsubvariants • not so aggressive as S/MA • diff. dg. from benign cysts !!!  biopsy (! inflammation) • treat.: luminal – simpleexcision x mural – acc. SMA • 4. metastasizing ameloblastoma • dg. in retrospect according to behaviour not histology!!; lung

  8. Ameloblastic carcinoma • 1. primary • rare, China • posterior mandible • Mi: malignant appearance A • lung metastases • 2. secondary (intra-, extraosseous) • A (long lasting)  AC • Mi: A + AC

  9. Squamous odontogenic tumor • benign x locally aggressive • extremely rare • M > F, ~ 40Y • mandible (from squamous nests in periodontal ligaments ?) • Mi: well-differentiated squamous epithelium + fibrous stroma • dif. dg.: squamous cell carcinoma squamous nests in wall of jaw cyst - RTG

  10. Calcifying epithelial odontogenic tumor • benign x locally aggressive • „Pindborg´s tumor“ (1955) • M ~ F, ~ 40Y; RTG: radiolucency + opacity • mandible (premolar/molar) • Mi: sheets of pleomorphic epithelial cells x mitoses absent amyloid  concentric calcifications • recurrence (20%) • treatment: according SMA • diff. dg.: poorly differentiated carcinoma

  11. Adenomatoid odontogenic tumor • benign, hamartoma ??? • M : F …1 : 2; peak ~ 15-30Y • anterior maxilla !!!; RTG: ~ odontogenic cyst • sometimes around crown of unerupted tooth • dif. dg.: follicular (dentigerous) cyst • Mi: solid nodules – epithelial cells nests, tubular structures + eosinophilic material calcifications • treatment: enucleation

  12. Ameloblastic fibroma • benign • rare, ~ 15Y • posterior mandible; RTG: uni- / multilocular cyst • Mi: ~ A + stroma ~ dental pulp • treatment: enucleation • diff. dg.: ameloblastoma – different treatment !!! • ---------------------------------------------------- • Ameloblastic fibrodentinom/fibroodontoma • developmental stages of odontoma

  13. Odontogenic sarcomas • 1. ameloblastic fibrosarcoma • malignant counterpart of ameloblastic fibroma • 2. ameloblastic fibrodentino-, fibroodonto-sarcoma • AFS + dentin / dentin + enamel • 1.+ 2. no prognostic significance

  14. Odontoma • true tumor (WHO), hamartoma ??? • most common odontogenic tumor • children, adolescents • treatment: enucleation • 1. complex type • posterior mandible, encapsulated • Mi: haphazardly enamel + dentin + cementum • 2. compound type • anterior maxilla, encapsulated • numerous tooth-like structures (odontoids)

  15. Dentinogenic ghost cell tumor •  solid variant of calcifying odontogenic cyst • canine-first molar area • locally aggressive

  16. Ghost cell odontogenic carcinoma • malignant counterpart of DGCT

  17. Odontogenic fibroma • benign, rare, somewhat controversial entity • F : M … 3 : 1, ~ 40Y • mandible : maxilla … 6.5 : 1 • Mi: epithelium-rich x epithelium-poor odontogenic epithelium + fibrous stroma • dif. dg.: dental follicle - RTG

  18. Odontogenic myxoma / myxofibroma • benign • 3rd most common odontogenic tumor • F > M, ~ 30Y • molar mandible • maxilla (maxillary sinus obliteration) • spread through medullar bone space • Mi: myxoid stroma + stellate cells • recurrence (25%) !!! • treatment: wide excision • dif. dg.: dental pulp tissue

  19. Cementoblastoma • benign • M ~ F, ~ 20Y • first molar of mandible • RTG: radiopaque mass + connection with tooth root !! • Mi: acellular cementum-like material bordered by plump cells without atypia fibrovascular tissue • dif. dg.: osteoblastoma, osteosarcoma • recurrence !!! • treatment: enucleation + tooth extraction

  20. Primary intraosseous squamous cell carcinoma • NO initial connection to oral mucosa • solid • from KOT • from other odontogenic cysts • metastases - LN and lungs

  21. Clear cell odontogenic carcinoma • WHO (1992) – benign x WHO (2005) - malignant • F > M, ~ 60Y • mandible • Mi: cells with clear cytoplasm + fibrous stroma • aggressive behaviour, recurrence • metastases – LN, lung, bone, … • t(12;22) … EWSR1-ATF1

  22. Melanotic neuroectodermal tumor of infancy • = melanotic progonoma, retinal anlage tumor, … • very rare (350 cases), neural crest ??? • infants (80% < 6th month, 95% < 1st year) • F : M …2 : 1 • maxilla (70%), mandible (10%), skull (10%) • rapidly growing pigmented mass, 3-4 cm • microscopy • small neuroblastic cells (granules), synaptophysin + • melanin-containing cells (melanosomes), CK, HMB45 + • local recurrence + metastases (7%) to LN, liver, bone

  23. Take home message • odontogenic tumors – rare x do exist • NOT only ameloblastoma • although benign x locally aggressive • DON´T rely on RTG itself • secondary inflammation may obscure the true nature of some lesions on microscopy • local recurrence (up to decades!) – long-term follow up !!! every lesion, incl. cysts, must be microscopically examined !!!

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