Ameloblastic fibrosarcoma in the maxilla, malignant transformation of ameloblastic fibroma

This report presents a fatal case of ameloblastic fibrosarcoma arising from an ameloblastic fibroma, originating in the maxilla of 19-year-old Japanese male. An analysis of previously reported fatal cases of ameloblastic fibrosarcoma is included. In the course of the disease, the mesenchymal component of ameloblastic fibroma showed a dramatic histopathological transformation into sarcoma following multiple recurrence and the patient died of uncontrollable local infiltration of the cranial base. Although many cases have seemed to show disappearance of the epithelial component as malignant transformation progressed, many benign appearing ameloblastoid epithelial masses were scattered throughout the sarcomatous area even in the fatal stage in the present case. No distant metastases were found at autopsy. During multiple recurrences of the lesion, a little dysplastic dentin which was closely associated with both epithelial and mesenchymal components was found, though it could not be observed in autopsy material. Ultrastructural findings in autopsy material showed that the mesenchymal component consisted of undifferentiated mesenchymal cells, fibroblastic and fibrocytic cells with marked cellular and nuclear pleomorphism and that the epithelial component closely resembled the enamel organ.