Neoplasms of the Oral and Maxillofacial Region

  • Román Carlos
  • Mario José Romañach


The objective of this chapter was to present specific histologic criteria for the diagnosis of the main tumors and pseudotumors of the jaws. Illustration of the histologic findings of these entities was provided in correlation with their clinical and radiographic manifestations, including histologic differential diagnoses. The histologic, clinical, and radiographic presentation of benign epithelial odontogenic tumors (ameloblastoma, calcifying epithelial odontogenic tumor, adenomatoid odontogenic tumor, and squamous odontogenic tumor), mixed odontogenic tumors (ameloblastic fibroma, ameloblastic fibro-odontoma, odontoameloblastoma, primordial odontogenic tumor, odontoma, and dentinogenic ghost cell tumor), and mesenchymal odontogenic tumors (odontogenic fibroma, odontogenic myxoma, and cementoblastoma) was offered. Two malignant odontogenic tumors (ameloblastic carcinoma and ameloblastic fibrosarcoma) were also considered. Additional tumors and pseudotumors affecting the gnathic bones included melanotic neuroectodermal tumor of infancy, juvenile solitary myofibroma of the jaws, extranodal T/NK lymphoma nasal type, rhinoscleroma (respiratory scleroma), and necrotizing sialometaplasia.


Ameloblastoma Calcifying epithelial odontogenic tumor Adenomatoid odontogenic tumor Primordial odontogenic tumor Dentinogenic ghost cell tumor Odontogenic myxoma Melanotic neuroectodermal tumor of infancy Juvenile solitary myofibroma Extranodal T/NK lymphoma nasal type 


  1. 1.
    El-Naggar AK, Chan J, Takata T, Grandis J, Slootweg P. WHO classification of tumours. Pathology and genetics of head and neck tumours. 4th ed. Lyon: IARC Press; 2017.Google Scholar
  2. 2.
    Philipsen HP, Reichart PA. Classification of odontogenic tumours: a historical review. J Oral Pathol Med. 2006;35:525–9.PubMedGoogle Scholar
  3. 3.
    Reegezi JA, Kerr DA. Odontogenic tumors: analysis of 706 cases. J Oral Surg. 1978;36:771–8.Google Scholar
  4. 4.
    Speight PM, Takata T. New tumour entities in the 4th edition of the World Health Organization classification of head and neck tumours: odontogenic and maxillofacial bone tumours. Virchows Arch. 2018;472(3):331–9. Epub 2017 Jul 3.PubMedPubMedCentralGoogle Scholar
  5. 5.
    Wright JM, Odell EW, Speight PM, Takata T. Odontogenic tumors, WHO 2005: where do we go from here? Head Neck Pathol. 2014;8(4):373–82.PubMedPubMedCentralGoogle Scholar
  6. 6.
    Wright JM, Vered M. Update from the 4th edition of the World Health Organization classification of head and neck Tumours: odontogenic and maxillofacial bone tumors. Head Neck Pathol. 2017;11:68–77.PubMedPubMedCentralGoogle Scholar
  7. 7.
    Woo S. Odontogenic Tumors. In: Oral pathology: a comprehensive atlas and text. Philadelphia: Elsevier/Saunders; 2012.Google Scholar
  8. 8.
    Neville BW, Damm DD, Allen CM, Chi AC. Oral and maxillofacial pathology. St. Louis: Elsevier Saunders; 2016.Google Scholar
  9. 9.
    Bilodeau EA, Collins BM. Odontogenic cysts and neoplasms. Surg Pathol Clin. 2017;10:177–222.PubMedGoogle Scholar
  10. 10.
    Mosqueda-Taylor A, et al. Odontogenic tumors in Mexico: a collaborative retrospective study of 349 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1997;84(6):672–5.PubMedGoogle Scholar
  11. 11.
    Martínez-Martínez M, Mosqueda-Taylor A, Carlos-Bregni R, Pires FR, Delgado-Azanero W, Neves-Silva R, Aldape-Barrios B, Paes-de AO. Comparative histological and immunohistochemical study of ameloblastomas and ameloblastic carcinomas. Med Oral Patol Oral Cir Bucal. 2017;22(3):e324–32.PubMedPubMedCentralGoogle Scholar
  12. 12.
    Ledesma-Montes C, Mosqueda-Taylor A, Carlos-Bregni R, de León ER, Palma-Guzmán JM, Páez-Valencia C, Meneses-García A. Ameloblastomas: a regional Latin-American multicentric study. Oral Dis. 2007;13(3):303–7.PubMedGoogle Scholar
  13. 13.
    Waldron CA, El-Mofty S. A histopathologic study of 116 ameloblastomas with special reference to the desmoplastic variant. Oral Surg Oral Med Oral Pathol. 1987;63:441–51.PubMedGoogle Scholar
  14. 14.
    Philipsen HP, Reichart PA. Unicystic ameloblastoma: a review of 193 cases from the literature. Oral Oncol. 1998;34:317–25.PubMedGoogle Scholar
  15. 15.
    Ackerman GL, Altini M, Shear M. The unicystic ameloblastoma: a clinicopathologic study of 57 cases. J Oral Pathol. 1988;17:541–6.Google Scholar
  16. 16.
    Philipsen HP, Reichart PA, Nikai H, et al. Peripheral ameloblastoma: biological profile based on 160 cases from the literature. Oral Oncol. 2001;37:17–27.PubMedGoogle Scholar
  17. 17.
    Hall JM, Weathers DR, Unni KK. Ameloblastic carcinoma: an analysis of 14 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2007;103:799–807.PubMedGoogle Scholar
  18. 18.
    Bologna-Molina R, Mikami T, Pereira-Prado V, Pires FR, Carlos-Bregni R, Mosqueda-Taylor A. Primordial odontogenic tumor: an immunohistochemical profile. Med Oral Patol Oral Cir Bucal. 2017;22(3):e314–23.PubMedPubMedCentralGoogle Scholar
  19. 19.
    Mosqueda-Taylor A, Carlos-Bregni R, Ramoa-Pires F, Aguirre-Urízar JM, de Almeida OP. Reply: primordial odontogenic tumour: it is truly novel. Histopathology. 2015;66(4):604–5.PubMedGoogle Scholar
  20. 20.
    Mosqueda-Taylor A, Pires FR, Aguirre-Urízar JM, Carlos-Bregni R, de la Piedra-Garza JM, Martínez-Conde R, Martínez-Mata G, Carreño-Álvarez SJ, da Silveira HM, de Barros Dias BS, de Almeida OP. Primordial odontogenic tumour: clinicopathological analysis of six cases of a previously undescribed entity. Histopathology. 2014;65(5):606–12.PubMedGoogle Scholar
  21. 21.
    Bologna-Molina R, Mikami T, Pereira-Prado V, Tapia-Repetto G, Pires FR, Carlos R, Mosqueda-Taylor A. Primordial odontogenic tumor: subepithelial expression of Syndecan-1 and Ki-67 suggests origin during early odontogenesis. Oral Dis. 2018;24(1–2):72–7.PubMedGoogle Scholar
  22. 22.
    Silva AR, Carlos-Bregni R, Vargas PA, de Almeida OP, Lopes MA. Peripheral developing odontoma in newborn. Report of two cases and literature review. Med Oral Patol Oral Cir Bucal. 2009;14(11):e612–5.PubMedGoogle Scholar
  23. 23.
    Philipsen HP, Reichart PA. Adenomatoid odontogenic tumour: facts and figures. Oral Oncol. 1999;35:125–31.PubMedGoogle Scholar
  24. 24.
    Philipsen HP, Reichart PA, Siar CH, Ng KH, Lau SH, Zhang X, Dhanuthai K, Swasdison S, Jainkittivong A, Meer S, Jivan V, Altini M, Hazarey V, Ogawa I, Takata T, Taylor AA, Godoy H, Delgado WA, Carlos-Bregni R, Macias JF, Matsuzaka K, Sato D, Vargas PA, Adebayo ET. An updated clinical and epidemiological profile of the adenomatoid odontogenic tumour: a collaborative retrospective study. J Oral Pathol Med. 2007;36(7):383–93.PubMedGoogle Scholar
  25. 25.
    Leon JE, Mata GM, Fregnani ER, Carlos-Bregni R, de Almeida OP, Mosqueda-Taylor A, Vargas PA. Clinicopathological and immunohistochemical study of 39 cases of Adenomatoid odontogenic tumour: a multicentric study. Oral Oncol. 2005;41(8):835–42.PubMedGoogle Scholar
  26. 26.
    Mosqueda-Taylor A, Carlos-Bregni R, Ledesma-Montes C, Fillipi RZ, de Almeida OP, Vargas PA. Calcifying epithelial odontogenic tumor-like areas are common findings in adenomatoid odontogenic tumors and not a specific entity. Oral Oncol. 2005;41(2):214–5.PubMedGoogle Scholar
  27. 27.
    Pindborg JJ. A calcifying epithelial odontogenic tumor. Cancer. 1958;11:838–43.PubMedGoogle Scholar
  28. 28.
    Franklin CD, Pindborg JJ. The calcifying epithelial odontogenic tumor: a review and analysis of 113 cases. Oral Surg Oral Med Oral Pathol. 1976;42:753–65.PubMedGoogle Scholar
  29. 29.
    Philipsen HP, Reichart PA. Calcifying epithelial odontogenic tumour: biological profile based on 181 cases from the literature. Oral Oncol. 2000;36:17–26.PubMedGoogle Scholar
  30. 30.
    Azevedo RS, Mosqueda-Taylor A, Carlos R, Cabral MG, Romañach MJ, de Almeida OP, Pires FR. Calcifying epithelial odontogenic tumor (CEOT): a clinicopathologic and immunohistochemical study and comparison with dental follicles containing CEOT-like areas. Oral Surg Oral Med Oral Pathol Oral Radiol. 2013;116(6):759–68.PubMedGoogle Scholar
  31. 31.
    Sánchez-Romero C, Carlos R, de Almeida OP, Romañach MJ. Microcystic calcifying epithelial odontogenic tumor. Head Neck Pathol. 2018;12(4):598–603.PubMedPubMedCentralGoogle Scholar
  32. 32.
    Rumayor A, Carlos R, Kirsch HM, de Andrade BA, Romañach MJ, de Almeida OP. Ghost cells in pilomatrixoma, craniopharyngioma, and calcifying cystic odontogenic tumor: histological, immunohistochemical, and ultrastructural study. J Oral Pathol Med. 2015;44(4):284–90.PubMedGoogle Scholar
  33. 33.
    Pullon PA, Shafer WG, Elzay RP, et al. Squamous odontogenic tumor: report of six cases of a previously undescribed lesion. Oral Surg Oral Med Oral Pathol. 1975;40:616–30.PubMedGoogle Scholar
  34. 34.
    Philipsen HP, Reichart PA. Squamous odontogenic tumor (SOT): a benign neoplasm of the periodontium. A review of 36 reported cases. J Clin Periodontol. 1996;23:922–6.PubMedGoogle Scholar
  35. 35.
    Wright JM. Squamous odontogenic tumor-like proliferations in odontogenic cysts. Oral Surg Oral Med Oral Pathol. 1979;47:354–8.PubMedGoogle Scholar
  36. 36.
    Trodahl JN. Ameloblastic fibroma: a survey of cases from the Armed Forces Institute of Pathology. Oral Surg Oral Med Oral Pathol. 1972;33:547–58.PubMedGoogle Scholar
  37. 37.
    Chen Y, Li T-J, Gao Y, et al. Ameloblastic fibroma and related lesions: a clinicopathologic study with reference to their nature and interrelationship. J Oral Pathol Med. 2005;34:588–95.PubMedGoogle Scholar
  38. 38.
    Cohen DM, Bhattacharyya I. Ameloblastic fibroma, ameloblastic fibro-odontoma, and odontoma. Oral Maxillofac Surg Clin North Am. 2004;16:375–84.PubMedGoogle Scholar
  39. 39.
    Takeda Y. Ameloblastic fibroma and related lesions: current pathologic concept. Oral Oncol. 1999;35:535–40.PubMedGoogle Scholar
  40. 40.
    Carlos-Bregni R, Mosqueda-Taylor A, Meneses-Garcia A. Ameloblastic fibrosarcoma of the mandible: report of two cases and review of the literature. J Oral Pathol Med. 2001;30:316–20.Google Scholar
  41. 41.
    Buchner A, Vered M. Ameloblastic fibroma: a stage in the development of a hamartomatous odontoma or a true neoplasm? Critical analysis of 162 previously reported cases plus 10 new cases. Oral Surg Oral Med Oral Pathol Oral Radiol. 2013;116(5):598–606.PubMedGoogle Scholar
  42. 42.
    Gupta DS, Gupta MK. Odontoameloblastoma. J Oral Maxillofac Surg. 1986;44:146–8.PubMedGoogle Scholar
  43. 43.
    Mosqueda-Taylor A, Carlos-Bregni R, Ramírez-Amador V, Palma-Guzmán JM, Esquivel-Bonilla D, Hernández-Rojase LA. Odontoameloblastoma. Clinico-pathologic study of three cases and critical review of the literature. Oral Oncol. 2002;38(8):800–5.PubMedGoogle Scholar
  44. 44.
    Budnick SD. Compound and complex odontomas. Oral Surg Oral Med Oral Pathol. 1976;42:501–6.PubMedGoogle Scholar
  45. 45.
    Philipsen HP, Reichart PA, Praetorius F. Mixed odontogenic tumours and odontomas. Considerations on interrelationship. Review of the literature and presentation of 134 new cases of odontomas. Oral Oncol. 1997;33(2):86–99.PubMedGoogle Scholar
  46. 46.
    Nelson BL, Thompson LDR. Compound odontoma. Head Neck Pathol. 2010;4:290–1.PubMedPubMedCentralGoogle Scholar
  47. 47.
    Tekkesin MS, Pehlivan S, Olgac V, et al. Clinical and histopathological investigation of odontomas: review of the literature and presentation of 160 cases. J Oral Maxillofac Surg. 2012;70:1358–61.Google Scholar
  48. 48.
    Allen CM, Hammond HL, Stimson PG. Central odontogenic fibroma WHO type: a report of 3 cases with an unusual associated giant cell reaction. Oral Surg Oral Med Oral Pathol. 1992;73:62–6.PubMedGoogle Scholar
  49. 49.
    Philipsen HP, Reichart PA, Sciubba JJ, et al. Odontogenic fibroma. In: Barnes L, Eveson JW, Reichart P, et al., editors. World Health Organization classification of tumours. Pathology and genetics of head and neck tumours. Lyon: IAARC Publishing Group; 2005. p. 315.Google Scholar
  50. 50.
    Mosqueda-Taylor A, Martínez-Mata G, Carlos-Bregni R, Vargas PA, Toral-Rizo V, Cano-Valdéz AM, Palma-Guzmán JM, Carrasco-Daza D, Luna-Ortiz K, Ledesma-Montes C, de Almeida OP. Central odontogenic fibroma: new findings and report of a multicentric collaborative study. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2011;112(3):349–58.PubMedGoogle Scholar
  51. 51.
    Eversole LR. Odontogenic fibroma, including amyloid and ossifying variants. Head Neck Pathol. 2011;5(4):335–43.PubMedPubMedCentralGoogle Scholar
  52. 52.
    Ledesma-Montes C, Gorlin RJ, Shear M, Prae Torius F, Mosqueda-Taylor A, Altini M, Unni K, Paes de Almeida O, Carlos-Bregni R, Romero de León E, Phillips V, Delgado-Azañero W, Meneses-García A. International collaborative study on ghost cell odontogenic tumours: calcifying cystic odontogenic tumour, dentinogenic ghost cell tumour and ghost cell odontogenic carcinoma. J Oral Pathol Med. 2008;37(5):302–8.PubMedGoogle Scholar
  53. 53.
    Martínez-Mata G, Mosqueda-Taylor A, Carlos-Bregni R, de Almeida OP, Contreras-Vidaurre E, Vargas PA, Cano-Valdéz AM, Domínguez-Malagón H. Odontogenic myxoma: clinico-pathological, immunohistochemical and ultrastructural findings of a multicentric series. Oral Oncol. 2008;44(6):601–7.PubMedGoogle Scholar
  54. 54.
    Lo Muzio L, Nocini PF, Favia G, et al. Odontogenic myxoma of the jaws: a clinical, radiologic, immunohistochemical, and ultrastructural study. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1996;82:426–33.PubMedGoogle Scholar
  55. 55.
    Noffke CEE, Raubenheimer EJ, Chabikuli NJ, et al. Odontogenic myxoma: review of the literature and report of 30 cases from South Africa. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2007;104:101–9.PubMedGoogle Scholar
  56. 56.
    Peltola J, Magusson B, Happonen RP, Borrman H. Odontogenic myxoma — a radiographic study of 21 tumors. Br J Oral Maxillofac Surg. 1994;32:298–302.PubMedGoogle Scholar
  57. 57.
    Martínez Martínez M, Mosqueda-Taylor A, Carlos R, Delgado-Azañero W, de Almeida OP. Malignant odontogenic tumors: a multicentric Latin American study of 25 cases. Oral Dis. 2014;20(4):380–5.PubMedGoogle Scholar
  58. 58.
    Christopher DM. Fletcher diagnostic histopathology of tumors. 4th ed. Philadelphia: Elsevier Saunders; 2013.Google Scholar
  59. 59.
    Al-Hakeem DA, Fedele S, Carlos R, Porter S. Extranodal NK/T-cell lymphoma, nasal type. Oral Oncol. 2007;43(1):4–14.PubMedGoogle Scholar
  60. 60.
    Sedano H, Carlos R. Respiratory Scleroma. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1996;81:665–771.PubMedGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Román Carlos
    • 1
  • Mario José Romañach
    • 2
  1. 1.Department of PathologyHospital Herrera-LlerandiGuatemala CityGuatemala
  2. 2.Department of Oral Diagnosis and PathologySchool of Dentistry, Federal University of Rio de JaneiroRio de JaneiroBrazil

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