Abstract
Ameloblastic fibrosarcoma (AFS) now designated as odontogenic sarcoma is a malignant odontogenic tumor characteristically composed of a benign epithelium and a malignant mesenchymal component. It can arise de novo without any preexisting lesion or it can result from the malignant transformation of ameloblastic fibroma (AF). Hereby, we report an extremely rare case of odontogenic sarcoma which was transformed from AF over a period of about 2 years. This is the first case to be reported after it has been reclassified as odontogenic sarcoma. A systematic review was also done to evaluate the studies that reported AFS arising de novo and AFS arising from AF. The objective of this study is to systematically review the studies that reported AFS arising de novo and AFS arising from AF. Articles that reported AFS arising de novo and AFS arising from AF were collected from PubMed, Medline, Embase, Cochrane, Google search and manual search. The results of the systematic review showed that six studies (46.1%) reported AFS arised de novo with no previous history of AF. Seven studies (53.84%) reported that amelobastic fibrosarcoma arised from AF. A rare case of odontogenic sarcoma transformed from AF is reported here. This is the first case report to be published on odontogenic sarcoma after the World Health Organization reclassification. AF once diagnosed should be treated immediately without any delay to avoid the chances of its malignant transformation into odontogenic sarcoma.