Cutaneous angiosarcoma (AS) is an aggressive malignant tumor. Chronic lymphedema is one of the major risk factor. Colled Stewart- Treves, It typically presents in women who develop lymphedema in the upper extremity secondary to axillary lymph node dissection for breast cancer surgery. We report a case of a 77-year-old women who underwent 12 year ago: a total mastectomy with an axillary lymph node dissection and an adjuvant chemotherapy and radiotherapy for left breast carcinoma, she presented 3 months ago edema on the left upper limb, Skin biopsy confirmed the diagnosis of angiosarcoma. A treatment by chemotherapy was proposed for the patient. Radical surgery is the best treatment to date for this rare disease. Conservative surgery with adjuvant radiotherapy is also possible. Systemic chemotherapy is reserved for locally advanced unresectable and metastatic forms. We advocate long term follow-up for every post mastectomy lymphedema to diagnosis this fatal disease when curable.
