NEUROFIBROMATOSIS TYPE-I AND MALIGNANCY - REVIEW OF 32 PEDIATRIC CASES TREATED AT A SINGLE INSTITUTION

Thirty-two cases of neurofibromatosis Type I (NF1) were identified amo ng 6,678 pediatric cancer patients treated at St. Jude Children's Rese arch Hospital over a 29-year period. A total of 35 malignant neoplasms have been diagnosed in these patients. Two of three patients with sec ond malignant neoplasms had colon cancer as the primary or second tumo r. Of particular interest are two cases in which both NF1 and malignan t peripheral nerve sheath tumors were present in multiple successive g enerations: a patient with colon cancer and non-Hodgkin lymphoma who h as a constitutional abnormality of the p53 gene, and a patient with ac ute lymphoblastic leukemia with the Philadelphia chromosome and other cytogenetic abnormalities, including the t(8;14). Outcome of patients in the largest subgroup, that of malignant peripheral nerve sheath tum ors, was favorable only for those patients having resectable extremity lesions. In contrast, all patients with central nervous system tumors are surviving. These cases reflect the molecular and cytogenetic abno rmalities that can be present in NF1 and the variety of tumors that ma y result in these patients. (C) 1994 Wiley-Liss, Inc.