A progressed case of Stewart–Treves syndrome in a patient with chronic idiopathic lymphedema in the lower extremity

Abstract

Angiosarcomas are uncommon malignant tumors that arise from vascular endothelial cells with poor prognosis. Stewart–Treves syndrome (STS) is a type of cutaneous angiosarcoma that develops in chronic lymphedema. Although the majority of lymphangiosarcomas arise from areas of lymphedema as a consequence of lymph node dissection in radical mastectomy, it has also occurred in other causes of lymphedema. In the pathophysiology of lymphangiosarcoma, impairment of the local immune response and induced angiogenesis are emphasized. A 69-year-old male patient with a previous diagnosis of chronic lymphedema in his left lower limb presented with STS and widespread metastases. The patient refused the treatment for lymphedema and ignored the lesion due to its innocuous appearance. In addition, during the presentation, he had Stage 4 disease and, therefore, he was not a candidate for curative operative treatment. Early tumor resection or limb amputation has the best outcomes in the treatment.

Keywords

• Chronic lymphedema
• lymphangiosarcoma
• metastatic angiosarcoma
• Stewart–Treves syndrome in lower extremity