Stewart-Treves syndrome: pathogenesis and management.

Stewart-Treves syndrome is a malignancy that arises within chronic lymphedema. Although classically described as a consequence of radical mastectomy, this lymphangiosarcoma has been documented to occur in cases of congenital and other causes of chronic secondary lymphedema. The development of this aggressive lymphangiosarcoma at sites of chronic lymphedema renders it a possible model for Kaposi sarcoma. Because of the increase in conservative treatment for breast carcinoma and improvement of operative and radiation therapy techniques, the prevalence of Stewart-Treves syndrome has decreased. Regardless, this malignancy significantly worsens patients' outcomes and needs to be diagnosed and treated early. Chemotherapy and radiation therapy have not improved survivorship significantly. Early amputation or wide local excision offers the best chance for long-term survival. Yet, overall prognosis remains dismal. Untreated patients usually live 5 to 8 months after diagnosis.