Stewart-Treves syndrome is a very rare lymphangiosarcoma with a poor prognosis. It usually develops in female patients after mastectomy with axillary lymph node dissection and is associated with chronic lymphedema. Stewart-Treves angiosarcoma is described in less than 1% of all soft tissue sarcomas, of which 0.5% occur in radically mastectomized patients. The case reported below describes the diagnosis and treatment of Stewart-Treves syndrome, with an initial clinical presentation of cutaneous lesions characteristic of the syndrome, manifesting ipsilaterally 22 years after quadrantectomy and radical right lymphadenectomy with adjuvant radiotherapy, the reported patient achieved, extraordinarily, a 12-year survival, far beyond the cases published to date.
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