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CA Cancer J Clin 1981; 31:333-335
doi: 10.3322/canjclin.31.6.333
© 1981 American Cancer Society
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CA: A Cancer Journal for Clinicians, Vol 31, 333-335, Copyright © 1981 by American Cancer Society

Neurofibrosarcoma at Irradiation Site in a Patient with Neurofibromatosis and Wilms' Tumor

Jen-Yih Chu M.D., Ph.D.1, Dennis M. O'Connor M.D.1, and Richard K. Danis M.D.2

1 Associate Professor, Department of Pediatrics-Adolescent Medicine, of St. Louis University School of Medicine and Cardinal Glennon Memorial Hospital for Children, in St. Louis, Missouri.
2 Associate Clinical Professor, Department of Surgery, of St. Louis University School of Medicine and Cardinal Glennon Memorial Hospital for Children, in St. Louis, Missouri.

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.






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Copyright © 1981 by American Cancer Society.