Primary Epithelioid Angiosarcoma of the Penis: A Case Report with a Brief Review of the Literature

Angiosarcoma of the penis is an exceptionally rare mesenchymal tumor, with only about 30 cases documented in the literature. Because of its rarity and the often nonspecific clinical presentation, histopathological examination plays a critical role in accurate diagnosis. Angiosarcoma of the penis typically arises in the corpus cavernosum but has also been reported in the glans and urethra, often presenting with metastases. This report details a case of epithelioid angiosarcoma of the penis in an 84-year-old man, who presented with a rapidly enlarging, violaceous nodule on the glans penis. Histopathology revealed atypical epithelioid endothelial cells with pleomorphism and mitotic activity, and immunohistochemical markers such as CD31, Erythroblast transformation-specific [ETS]-related gene, and cellular myelocytomatosis oncogene confirmed the malignant endothelial neoplasm. The differential diagnosis excluded conditions such as epithelioid hemangioendothelioma and Kaposi sarcoma. Because of the rarity, standardized treatment protocols are lacking; however, wide excision with tumor-free margins and the potential for targeted therapies based on gene amplification are considered important. The review highlights the diagnostic challenges, prognostic factors, and current therapeutic approaches, emphasizing the need for further research to develop standardized treatment protocols.