A case of Stewart-Treves syndrome suspected of being epithelioid angiosarcom

Abstract

An 80-year-old Japanese woman presented with a 1-month history of violaceous, hemorrhagic nodules, and purpura on her right leg. We first diagnosed her with Stewart-Treves syndrome by angiosarcoma based on her medical history of surgery and irradiation for cervical cancer and chronic bilateral lymphedema of the legs. Histopathological examination demonstrated diffuse epithelioid proliferation of atypical tumor cells, some of which contained intracytoplasmic vacuoles and red blood cells. The tumor cells were diffusely immunoreactive for D2-40 and tested negative for other stains. After the diagnosis of epithelioid angiosarcoma, we administered irradiation and chemotherapy containing paclitaxel ; however, she died because of hemorrhagic pneumothorax by lung metastases of epithelioid angiosarcoma. Epithelioid angiosarcomas are rare variants of angiosarcoma that mainly originate in soft tissues. Our case demonstrated unusual pathological characteristics of epithelioid angiosarcoma. Variant types of angiosarcoma and other undifferentiated sarcomas should be included among the differential diagnoses of tumors in Stewart-Treves syndrome.[Skin Cancer (Japan) 2018 ; 33 : 154-157]