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

A ngiosarcoma 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 StewarteTreves 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. This is supported by recent research showing that these tumors feature a characteristicMYC amplification, a nuclear transcription factor crucial for vascular endothelial growth factoredependent angiogenesis. We report a case of angiosarcoma arising in a chronically edematous limb of a patient with a spinal cord injury (SCI). We report this unusual case to highlight angiosarcoma as a potential complication of lymphedema in the SCI population. With the number of traumatic SCIs on the rise, these patients represent a subset of the population who are uniquely susceptible to developing lymphedema and its complications. Management of these patients is complex, often requiring a multidisciplinary approach.