Neurofibrosarcoma at irradiation site in a patient with neurofibromatosis and Wilms' tumor.

A female patient with neurofibromatosis had nephrectomy performed because of Wilms' tumor at the age of four and a half. She received radiation therapy and chemotherapy (actinomycin D) after surgery. She had subsequent local recurrence and lung metastasis, which were surgically excised and successfully treated with additional radiation therapy and chemotherapy (vincristine and actinomycin D). However, neurofibrosarcoma at the irradiation site developed seven years after radiation therapy. She died 22 months later because of recurrence and metastasis of neurofibrosarcoma. Radiation therapy's association with malignant transformation of neurofibroma is discussed.