Stewart-Treves syndrome: a case report of lymphedema-related angiosarcoma

Abstract

Stewart-Treves syndrome (STS) is an angiosarcoma associated with lymphedema deriving more often from radical mastectomy and longstanding lymphedema, first described in 1948. Irradiation is also commonly associated with chronic lymphedema of extremities. It generally occurs about 10 years after the mastectomy and/or radiotherapy. The prognosis is very poor with a mean survival of 20 months from the diagnosis especially when radical surgery is not possible. We present the case of STS in an 89-year-old female who underwent left upper outer quadrantectomy for invasive ductal carcinoma in 2009 followed by axillary lymphadenectomy, radiotherapy, and hormonal therapy until 2014. She presented swollen upper left limb associated with hemorrhagic red papular lesions. Skin biopsy revealed the presence of lymphedema-associated angiosarcoma. Radical surgery was not possible, so she underwent conservative therapy with pazopanib, a tyrosine kinase inhibitor, with benefit. Long-term follow-up of these patients is crucial to intercept this condition at an early stage.