Objective: Epithelioid angiosarcoma of the vagina is a rare variant that can easily be misdiagnosed considering the much higher frequency of epithelial neoplasms at that particular site.
Material and Methods: We report the case of a 41-year-old gravida 2, para 1, aborta 1, with no prior history of irradiation, who consulted after the discovery of 3 lesions at the lower right portion of the vagina.
Result: The lesion consisted of epithelioid cells with high-grade nuclei and prominent nucleoli. These cells expressed CD31, CD34, factor VIII, Fli-1, vimentin, smooth muscle actin, and WT-1. Keratin 8/18 was focally positive. They were immunonegative for keratin AE1/AE3, keratin 34βE12, keratin 7, keratin 20, S100, HMB-45, myogenin, desmin, and human herpesvirus type 8. Polymerase chain reaction–based HPV viral search was also negative.
Conclusions: A broad immunohistochemical panel including antibodies against vascular differentiation markers as well as various cytokeratins allows proper diagnosis of this unusual and aggressive entity.