Abstract

Stewart–Treves syndrome, also known as cutaneous angiosarcoma, refers to a lymphangiosarcoma, a rare complication that forms as a result of chronic, long-standing lymphedema. Although it most commonly refers to malignancies associated with chronic lymphedema resulting from mastectomy and/or radiotherapy for breast cancer, it may also describe lymphangiosarcomas that result from congenital and other causes of chronic secondary lymphedema. Lymphangiosarcoma arising from cancer-related lymphedema has become much less common with better surgical techniques, radiation therapy, and conservative treatment. The prognosis, even with wide surgical excision and subsequent radiotherapy, is poor.

Treatment

The treatment of choice is a large resection or amputation of the affected limb. Radiation therapy can precede or follow surgical treatment. Tumors that have advanced locally or have metastasized can be treated with mono or polychemotherapy, systemically or locally. However, chemotherapy and radiation therapy have not been shown to improve survivorship significantly.

Prognosis

Early detection is key. Untreated patients usually live 5 to 8 months after diagnosis.

Incidence

In the 1960s, the incidence 5 years after a radical mastectomy varied from 0.07% to 0.45%.

Today, it occurs in 0.03% of patients surviving 10 or more years after radical mastectomy.