Stewart–Treves syndrome in a spinal cord injury patient with MYC amplification

Angiosarcoma is a rare, aggressive vascular malignancy with a poor prognosis. Primary cutaneous angiosarcoma arises de novo on the head and neck of the elderly, whereas secondary angiosarcoma arises in the setting of known risk factors, including chronic lymphedema or previous irradiation. When associated with chronic lymphedema, it is eponymously referred to as Stewart–Treves syndrome. The pathophysiology of tumorigenesis in these cases is not fully understood, but may be related to ongoing angiogenesis and impaired cellular immunity induced by lymphedema.