Oral presentation of epithelioid angiosarcoma with first sign in the scapula: report of a case and review of the literature.

Abstract

Occurrence of a primary or metastatic angiosarcoma in the oral cavity is extremely rare. The term "epithelioid angiosarcoma" (EA) has been used to designate a morphological variant of angiosarcoma characterized by poorly differentiated epithelial-like cells arranged in carcinoma-like fashion, but which still forms identifiable vascular channels. To our knowledge, EA in the oral region is extremely rare. Only two previous instances of EA in the maxilla have been reported. We present an additional oral case of EA in a 71-year-old man. Histology of the initial oral biopsy revealed suspicion of un-differentiated carcinoma. In order to confirm the diagnosis, immunohistochemical examinations were performed. The final diagnosis was EA. The patient died of multiple metastases shortly after the final diagnosis, implying an aggressive clinical course. This case showed that it was essential to use the vascular markers, such as FVIII-Rag and CD34, for a correct histological diagnosis of EA. The oral EA described here almost certainly represents a metastatic focus, rather than the primary site of tumor origin. This is because clinical history of EAs appears to arise in deep, rather than in more superficial tissues.