Ameloblastoma

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Definition
The most common epithelial odontogenic neoplasm of low malignant potential divided into several subtypes including follicular, plexiform, granular cell, desmoplastic, vascular, acanthomatous, papilliferous-keratotic and with dentinoid induction.

Clinical Features

Comparatively rare: o 1% of tumors and cysts arising in jaws Most common: o epithelial odontogenic tumor1 o in third to fifth decades2,3 No gender or population preference >80% in mandible: o 70% of these in molar/ramus area Duration: o few weeks to 50 years4,5 (Figs 14

Fig. 1: First of four radiographs (Figures 14) illustrating history of ameloblastoma associated with impacted third molar tooth in a 19-year-old female patient. April, 1971. Subtle, atypical radiolucency can be discerned below tooth. Tooth was surgically removed. No surgical specimen was obtained. (Courtesy of Dr. William Randall and Dr. Clark Borstad, Minneapolis.)

Fig. 2: Second of four radiographs (Figures 14) illustrating history of ameloblastoma associated with impacted third molar tooth in a 19-year-old female patient. September, 1974. Routine dental roentgenogram revealed residual or recurrent cystic lesion. Surgical curettage was performed and early ameloblastoma demonstrated. No further treatment was given. (Courtesy of Dr. William Randall and Dr. Clark Borstad, Minneapolis.)

Fig. 3: Third of four radiographs (Figures 14) illustrating history of ameloblastoma associated with impacted third molar tooth in a 19-year-old female patient. February, 1978. Multilocular radiolucency of more typical character. Tumor was resected. (Courtesy of Dr. William Randall and Dr. Clark Borstad, Minneapolis.)

Fig. 4: Fourth of four radiographs (Figures 14) illustrating history of ameloblastoma associated with impacted third molar tooth in a 19-year-old female patient. December, 1978. There is no evidence of residual tumor. (Courtesy of Dr. William Randall and Dr. Clark Borstad, Minneapolis.) ) Spread and Metastasis

Borderline (low-grade malignant) category: o invasive properties o tendency to recur o rarely, distant metastases:

o o

especially to lungs also to CNS611

Pathogenesis

May arise from: o epithelial lining of dentigerous cyst o remnants of dental lamina and enamel organ o basal layer of oral mucosa

Gross Pathology

Traditional division into solid and (multi)cystic types arbitrary Usually some cystic change (Fig. 5

Fig. 5: Gross appearance of ameloblastoma of mandible. )

Sometimes multicentric1216

Histopathology

Many subtypes or patterns: o including: o follicular: o a predominant pattern (together with plexiform) o attempt to mimic dental organ epithelium o outermost cells resemble inner dental epithelium of developing tooth follicle (i.e. ameloblastic layer): o tall columnar o polarization of nuclei away from basement membrane17 o central portion of epithelial island:

o o

loose network of cells resembling stellate reticulum: squamous metaplasia within stellate reticulum gives rise to acanthomatous type (Fig. 6

Fig. 6: Granular cell ameloblastoma. Most of the tumor cells have an abundant, deeply granular cytoplasm. )
o o o o o o o o

epithelial islands demonstrate little inductive influence on fibrous connective tissue stroma plexiform: a predominant pattern (together with follicular) irregular masses and interdigitating cords of epithelial cells minimum of stroma:17,18 occasionally contains multinucleated osteoclastic-type giant cells19 acanthomatous (see Fig. 6) papilliferous keratotic (Fig. 7

Fig. 7: Keratotic ameloblastoma. Ameloblastic epithelium surrounds large keratin-filled cavities. )

granular cell desmoplastic vascular with dentinoid induction (dentinoameloblastoma)2026 two or more may coexist little evidence to suggest that one is more aggressive than another Enamel and dentin are not formed Electron microscopy: o tumor cells: o clearcut evidence of epithelial differentiation in form of: o bundles of tonofilaments o complex desmosomes o some differences between follicular and plexiform types2729 o granular variety shows same type of lysosomal formations as in ordinary granular cell tumor30,31
o o o o o o

Special Stains and Immunohistochemistry

Tumor cells: o strong reactivity for keratin o surrounded by continuous layer of laminin: o pattern similar to that in developing tooth32 o express major keratins: o CK5 o CK14 Cells in stellate reticulum-like areas: o coexpression of CKs 8, 18, and 1933 Calretinin positive: o most solid and multicystic ameloblastomas (as well as unicystic)34 o almost always restricted to stellate reticulum-like epithelium: o may be diagnostic aid for identification of these tumors Granular cell ameloblastoma: o keratin positive and S-100 protein negative o consistent with ameloblastic nature o in contrast to ordinary granular cell tumor o CD68 positive: o like ordinary granular cell tumor o indicates that granular cell change due to lysosomal overload35

Diagnosis

Made by fine needle aspiration36 Malignant ameloblastoma: o term accepted by WHO for metastasizing tumors that retain typical morphology of ameloblastoma37 (as opposed to ameloblastic carcinoma)

usually distant metastases have been preceded by several local recurrences38

Other Investigations

Radiography: o usually lytic expansile lesion

Differential Diagnosis
Select up to 2 differential diagnoses to compare with Ameloblastoma Ameloblastic Carcinoma [Mandible and Maxilla] (View full diagnosis) Clear Cell Odontogenic Carcinoma [Mandible and Maxilla] (View full diagnosis) Calcifying Epithelial Odontogenic Tumor [Mandible and Maxilla] (View full diagnosis) Keratinizing and Calcifying Odontogenic Cyst [Mandible and Maxilla] (View full diagnosis)
Compare Differentials

Focal reactive hyperplasia in a dentigerous cyst vs unicystic ameloblastoma: o unicystic ameloblastoma represents either: o malignant ameloblastic transformation to a dentigerous cyst o a predominantly cystic form of ameloblastoma o minimal criterion for diagnosis of ameloblastoma is palisaded basal layer with stellate reticulum-like epithelium above3942 (Fig. 8

Fig. 8: Unicystic ameloblastoma fulfilling the minimum criteria for this entity, which should be distinguished from reactive epithelial changes in a radicular cyst. ):
o

immunoreactivity for calretinin supports diagnosis43

Management

Enucleation: o usually curative: o may be local recurrence: o particularly if ameloblastic islands embedded in cyst wall4446

References
1 Larsson , Almrn H. Ameloblastoma of the jaws. Acta Pathol Microbiol Scand [A]. 1978;86:337349. 2 Daramola JO, Ajaglae HA, Oluwasanmi JO. Ameloblastoma of the jaws in Nigerian children. A review of sixteen cases. Oral Surg Oral Med Oral Pathol. 1975;40:458463. 3 Sehdev MK, Huvos AG, Strong EW, Gerold FP, Willis GW. Ameloblastoma of maxilla and mandible. Cancer. 1974;33:324333. 4 Hoffman PJ, Baden E, Rankow RM, Potter GD. Fate of uncontrolled ameloblastoma. Oral Surg Oral Med Oral Pathol. 1968;26:419426. 5 Mehlisch DR, Dahlin DC, Masson JK. Ameloblastoma. A clinicopathologic report. J Oral Surg. 1972;30:922. 6 Gorlin RJ. Odontogenic tumors. Gorlin RJ, Goldman HM editor. Thoma's oral pathology. ed. 6. St. Louis: Mosby; 1970. 7 Hoke HF, Harrelson AB. Granular cell ameloblastomas with metastases to cervical vertebrae. Cancer. 1967;20:991999. 8 Ikemura K, Tashiro H, Fujino H, Ohbu D, Nakajima K. Ameloblastoma of the mandible with metastasis to the lungs and lymph nodes. Cancer. 1972;29:930940. 9 Madiedo G, Choi H, Kleinman JG. Ameloblastoma of the maxilla with distant metastases and hypercalcemia. Am J Clin Pathol. 1981;75:585591.

10 Phillips SD, Corio RL, Brem H, Mattox D. Ameloblastoma of the mandible with intracranial metastasis. A case study. Arch Otolaryngol Head Neck Surg. 1992;118:861 863. 11 White RM, Patterson JW. Distant skin metastases in a long-term survivor of malignant ameloblastoma. J Cutan Pathol. 1986;13:383389. 12 Gardner DG. Peripheral ameloblastoma. A study of 21 cases, including 5 reported as basal cell carcinoma of the gingiva. Cancer. 1977;39:16251633. 13 Gorlin RJ. Odontogenic tumors. Gorlin RJ, Goldman HM editor. Thoma's oral pathology. ed. 6. St. Louis: Mosby; 1970. 14 Richardson JF, Greer RO. Ameloblastoma of mucosal origin. Arch Otolaryngol. 1974;100:174175. 15 Vickers RA, Gorlin RJ. Ameloblastoma. Delineation of early histopathologic features of neoplasia. Cancer. 1970;26:699710. 16 Wesley RK, Borninski ER, Mintz S. Peripheral ameloblastoma. Report of a case and review of the literature. J Oral Surg. 1977;35:670672. 17 Kramer IRH, Pindborg JJ, Shear M. Histological typing of odontogenic tumours, ed. 2. In World Health Organization: International Histological Classification of Tumours. Berlin: Springer-Verlag; 1992;. 18 Gorlin RJ. Odontogenic tumors. Gorlin RJ, Goldman HM editor. Thoma's oral pathology. ed. 6. St. Louis: Mosby; 1970. 19 Richard BM, Thyveetil M, Sharif H, Athanasou NA. Ameloblastoma with stromal multinucleated giant cells. Histopathology. 1994;25:497500. 20 Altini M, Slabbert HD, Johnston T. Papilliferous keratoblastoma. J Oral Pathol Med. 1991;20:4648. 21 Burkes EJ, Wallace DA. Granular cell ameloblastoma. J Oral Surg. 1976;34:742744. 22 Collini P, Zuchini N, Vessecchia G, Guzzo M. Papilliferous keratoameloblastoma of mandible: a papillary ameloblastic carcinoma. Report of a case with a 6-year follow-up and review of the literature. Int J Surg Pathol. 2002;10:149155. 23 Hartman KS. Granular-cell ameloblastoma. Oral Surg Oral Med Oral Pathol. 1974;38:241243. 24 Higuchi Y, Nakamura N, Ohishi M, Tashiro H. Unusual ameloblastoma with extensive stromal desmoplasia. J Craniomaxillofac Surg. 1991;19:323327.

25 Nasu M, Takagi M, Yamamoto H. Ultrastructural and histochemical studies of granular-cell ameloblastoma. J Oral Pathol. 1984;13:448456. 26 Slabbert H, Altini M, Crooks J, Uys P. Ameloblastoma with dentinoid induction: Dentinoameloblastoma. J Oral Pathol Med. 1992;21:4648. 27 Larsson , Almrn H. Ameloblastoma of the jaws. Acta Pathol Microbiol Scand [A]. 1978;86:337349. 28 Mehlisch DR, Dahlin DC, Masson JK. Ameloblastoma. A clinicopathologic report. J Oral Surg. 1972;30:922. 29 Nasu M, Ishikawa G. Ameloblastoma. Light and electron microscopic study. Virchows Arch [A]. 1983;399:163175. 30 Navarrete AR, Smith M. Ultrastructure of granular cell ameloblastoma. Cancer. 1971;27:948955. 31 Tandler B, Rossi EP. Granular cell ameloblastoma. Electron microscopic observations. J Oral Pathol. 1977;6:401412. 32 Thesleff I, Ekblom P. Distribution of keratin and laminin in ameloblastoma. Comparison with developing tooth and epidermoid carcinoma. J Oral Pathol. 1984;13:8596. 33 Vigneswaran N, Whitaker SB, Bodnick SD, Waldron CA. Expression patterns of epithelial differentiation antigens and lectin-binding sites in ameloblastomas: A comparison with basal cell carcinomas. Hum Pathol. 1993;24:4967. 34 Altini M, Coleman H, Doglioni C, Favia G, Maiorano E. Calretinin expression in ameloblastomas. Histopathology. 2000;37:2732. 35 Dina R, Marchetti C, Vallania G, Corinaldesi G, Eusebi V. Granular cell ameloblastoma an immunocytochemical study. Pathol Res Pract. 1996;192:541546. 36 Radhika S, Nijhawan R, Das A, Dey P. Ameloblastoma of the mandible: Diagnosis by fine-needle aspiration cytology. Diagn Cytopathol. 1993;9:310313. 37 Slootweg PJ, Mller H. Malignant ameloblastoma or ameloblastic carcinoma. Oral Surg Oral Med Oral Pathol. 1984;57:168176. 38 Kunze E, Donath K, Luhr HG, Engelhardt W, De Vivie R. Biology of metastasizing ameloblastoma. Pathol Res Pract. 1985;180:526535. 39 Gardner DG, Corio RL. The relationship of plexiform unicystic ameloblastoma to conventional ameloblastoma. Oral Surg Oral Med Oral Pathol. 1983;56:5460.

40 Leider AS, Eversole LR, Barkin ME. Cystic ameloblastoma. A clinicopathologic analysis. Oral Surg Oral Med Oral Pathol. 1985;60:624630. 41 McMillan MD, Smillie AC. Ameloblastomas associated with dentigerous cysts. Oral Surg Oral Med Oral Pathol. 1981;51:689696. 42 Vickers RA, Gorlin RJ. Ameloblastoma. Delineation of early histopathologic features of neoplasia. Cancer. 1970;26:699710. 43 Coleman H, Altini M, Ali H, Doglioni C, Favia G, Maiorano E. Use of calretinin in the differential diagnosis of unicystic ameloblastomas. Histopathology. 2001;38:312 317. 44 Gardner DG. Plexiform unicystic ameloblastoma. A diagnostic problem in dentigerous cysts. Cancer. 1981;47:13581363. 45 Gardner DG, Corio RL. Plexiform unicystic ameloblastoma. A variant of ameloblastoma with a low-recurrence rate after enucleation. Cancer. 1984;53:17301735. 46 Li T-J, Wu Y-T, Yu S-F, Yu G-Y. Unicystic ameloblastoma: a clinicopathologic study of 33 chinese patients. Am J Surg Pathol. 2000;24:13851392.