Ameloblastic fibrosarcoma – A rarity?

Ameloblastic fibrosarcoma (AFS) is a rare malignant odontogenic tumour classified by the WHO (1992) as an odontogenic sarcoma and defined as “a neoplasm with a similar structure to ameloblastic fibroma (AF) but in which the ectomesenchymal component shows the features of a sarcoma.” The first report of AFS was published by Heath in 1887. AFS and related lesions are less frequently diagnosed than odontogenic carcinomas. Approximately two-thirds of AFSs seem to arise de novo, but others have developed in recurrent AF, in which the ectomesenchymal cells retain their embryonic appearance and develop malignant characteristics. We report a rare case of an aggressive odontogenic neoplasm, the incisional biopsy of which showed the features of AF, while the excisional biopsy revealed the features of malignancy, suggestive of AFS. The purpose of this report is to discuss the diagnostic difficulties, whether AFS is truly an extremely rare tumour as reported earlier and, lastly, should the treatment protocols of AFs be revised, as 44% of AFSs arise from recurrent AFs.