Stewart-Treves Syndrome: A Rare But Aggressive Complication of Breast Cancer-Related Lymphedema

Stewart-Treves syndrome (STS) is an angiosarcoma that usually develop in an extremity with longstanding lymphedema. Most affected patients have a history of breast cancer treated with radical mastectomy. Here, we report a case of STS with breast cancer-related lymphedema (BCRL) for a period of seven years. A 56-year-old woman presented with chronic lymphedema of the left arm. Nine years previously she had modified radical mastectomy for grade 2, invasive, ductal breast cancer. Upon physical examination, a tender, purplish lesion on the medial half of the affected arm was observed. The lesion spread rapidly with different-sized, scattered, purple-colored lesions in the affected area. A prompt skin biopsy was reported as STS. An immediate arm amputation was performed. However, a few months later she presented with new lesions on the anterior thorax and subsequent local recurrence around the scar. She received radiation-therapy. However, six months later the angiosarcoma had spread to the pelvic and upper limb area with scattered skin lesions. She had several problems during the chemotherapy and radiation-therapy, although she survived beyond 20 months. In conclusion, STS is a rare but aggressive and important complication of BCRL. Awareness of rapidly progressing skin lesions and detailed investigation, as well as prompt surgery is necessary for patients with BCRL in order to relatively increase the survival time.