Angiosarcoma developing in a patient with Neurofibromatosis (von Recklinghausen's disease)

The authors present an angiosarcoma of the scrotum and penis. The disease occurred in a 61-year-old man with a long history of neurofibromatosis (von Recklinghausen's disease). The histologic and ultrastructural features are described. The authors speculate that the angiosarcoma, which developed in a location without histologic evidence of neurofibromatosis, possibly represents an expression of an abnormal genome in vascular tissue. The angiosarcoma responded to radiation therapy only temporarily, recurred after surgery, and did not respond to Adriamycin, dactinomycin or cis-platinum. The patient died three years after the onset of his angiosarcoma.