Stewart-Treves syndrome – case report and review of the literature

Introduction. Chronic lymphedema, mainly due to total mastectomy with axillary lymph nodes dissection for breast cancer, can induce development of rare and highly malignant cutaneous angiosarcoma. This condition is called Stewart-Treves syndrome (STS). There are only about 400 cases of STS reported in the literature. The long-term prognosis is poor; 5-year survival is up to 10%. Limb amputation appears to be the treatment of choice.

Objective. To present a case of Stewart-Treves syndrome with a fatal outcome, which developed 12 years after mastectomy with lymphadenectomy.

Case report. A 65-year-old woman was admitted to our department for confluent purpuric macules, hemorrhagic blisters, and violaceous-black nodules, involving the whole left arm and forearm, with chronic severe lymphedema of the extremity. She had a history of left-sided breast cancer 12 years previously, treated with mastectomy with excision of axillary lymph nodes; subsequently, the failure of lymph drainage and lymph stasis developed. Histopathological examination of the tumor was consistent with angiosarcoma. The patient was disqualified from a surgical procedure due to the late stage of malignancy and died approximately a month later.

Conclusions. Given unsatisfactory treatment options, the prevention of long-lasting lymphedema is crucial to improve prognosis of patients with breast cancer.