Angiosarcoma secondary to primary upper limb lymphedema

Abstract

Stewart–Treves syndrome (STS) is a rare entity with a poor prognosis defined as an angiosarcoma appearing in a specific clinical setting, in 90% of cases in a context of breast neoplasia. Herein, we report a rare case of STS of the upper limb as a complication of primary lymphedema. A 55-year-old male was referred for a large, reddish, necrotic multinodular tumor of rapid extension on the anterior aspect of the right forearm. A biopsy of the lesions was performed, confirming the diagnosis of angiosarcoma. The evaluation of tumor extension showed no distant metastasis. An amputation of the arm was performed. Angiosarcoma as a complication of primary lymphedema is a very rare entity with very poor prognosis.

Keywords

• angiosarcoma
• lymphedema
• stewart–treves syndrome