Stewart-Treves syndrome. Report of one case

Abstract

Angiosarcomas are malignant tumors derived from the endothelium of blood vessels (hemangiosarcomas) or lymph vessels (lymphangiosarcomas). Lymphedema of the limbs is considered secondary when extrinsic injuries are observed and primary when these injuries are not present. Stewart-Treves syndrome or a Lymphangiosarcoma, developed over a chronic lymphedema, is a rare complication described in mastectomized patients but it can be observed in lymphedemas located elsewhere. It appears as nodular skin lesions that grow, multiply quickly and frequently metastasize. We report a 40-year-old male with an angiosarcoma associated with primary chronic lower limb lymphedema. The patient consulted for a history of weight loss and malaise and appearance of violaceous lesions over the zone of lymphedema and inguinal lymph node involvement. A CT scan showed bilateral lung lesions and enlargement of inguinal and iliac lymph nodes. A biopsy of one of the skin lesions disclosed an angiosarcoma, diagnosis that was confirmed with immunohistochemistry. Chemotherapy was started but the patient died five months after the diagnosis.