Angiosarcoma in a chronically immunosuppressed renal transplant recipient: report of a case and review of the literature.

Angiosarcomas are rare malignant tumors of endothelial origin that occur predominantly in the adult population. To date, only 12 cases have been described after renal transplantation. We review the literature and present an additional case of a 59-year-old patient in whom lower-extremity lesions of cutaneous angiosarcoma developed several years after transplantation. Of the 13 patients, clinical details were available in only 12 cases. There were 10 male and 2 female patients. The mean age at diagnosis was 47.6 years (age range, 28-71 years). Almost all patients were on a combination immunosuppressive regimen, and the interval between transplantation and the development of the tumor was variable in the range of 8 months to 12 years. In five patients, the tumor arose within or adjacent to an arteriovenous fistula site. Cutaneous involvement was observed in only four cases. The histologic spectrum consisted of both well-differentiated and poorly differentiated tumors. In one patient, features of the hemophagocytic syndrome were clinically present and the tumor morphologically displayed prominent erythrophagocytosis. In two of the cases, including the patient described in the current report, polymerase chain reaction was performed on lesional tissue for human herpesvirus-8 and the result was negative for the presence of viral DNA. Despite the use of multimodal aggressive therapy in most patients, death as a result of disease dissemination occurred over variable time periods (range, 1-15 months).