Angiosarcoma complicating systemic sclerosis: a case report.

Cutaneous angiosarcoma is a rare malignant vascular tumor with a poor prognosis, most commonly affecting elderly white men. Diagnosis usually is delayed because the tumor has a highly variable and often innocuous presentation. Cutaneous angiosarcoma has been associated with a number of conditions and factors, including chronic lymphedema, prior radiation therapy, exposure to chemicals, and vascular malformations. We report the case of a 40-year-old black man with systemic sclerosis (SSc) who developed a cutaneous angiosarcoma in an area of sclerodermatous scalp. We propose that vascular endothelial growth factor (VEGF) overexpression in sclerodermatous skin may predispose a patient to the development of vascular tumors, such as angiosarcoma. Because early diagnosis and treatment positively impact survival outcome in patients with angiosarcoma, it is essential that physicians recognize the association of angiosarcoma and SSc and maintain a low threshold for performing a biopsy when suspicious lesions are present on sclerodermatous skin.