Epithelioid angiosarcoma of the vulva: A case report

Angiosarcoma is a rare malignancy of lymphatic or vascular endothelium that can arise anywhere in the body (Young et al., 2010). Angiosarcomas make up approximately 2% of soft tissue and 5.4% of cutaneous sarcomas. Chronic lymphedema and radiation treatment are each known risk factors for development of angiosarcoma, which has been well described primarily in patients treated for breast cancer (Huang and Mackillop, 2001). Lymphangiosarcoma occurring secondary to chronic lymphedema in patients who had undergone mastectomy was first reported in 1948 and is now known as Stewart-Treves syndrome (Stewart and Treves, 1948). Angiosarcomas of gynecologic origin are rare, with only 4 reported in the literature. We present a case of vulvar epithelioid angiosarcoma associated with radiation and chronic lymphedema.