Neurofibromatosis type I and malignancy: Review of 32 pediatric cases treated at a single institution

Patricia Shearer, David Parham, Edward Kovnar, Larry Kun, Bhaskar Rao, Thom Lobe, Charles Pratt

Research output: Contribution to journalArticle

59 Scopus citations

Abstract

Thirty‐two cases of neurofibromatosis Type I (NF1) were identified among 6,678 pediatric cancer patients treated at St. Jude Children's Research Hospital over a 29‐year period. A total of 35 malignant neoplasms have been diagnosed in these patients. Two of three patients with second malignant neoplasms had colon cancer as the primary or second tumor. Of particular interest are two cases in which both NF1 and malignant peripheral nerve sheath tumors were present in multiple successive generations: a patient with colon cancer and non‐Hodgkin lymphoma who has a constitutional abnormality of the p53 gene, and a patient with acute 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 tumors, 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 abnormalities that can be present in NF1 and the variety of tumors that may result in these patients. © 1994 Wiley‐Liss, Inc.

Original languageEnglish (US)
Pages (from-to)78-83
Number of pages6
JournalMedical and Pediatric Oncology
Volume22
Issue number2
DOIs
StatePublished - 1994

Keywords

  • cancer
  • malignancies
  • novel cases in children with NF1
  • outcome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Oncology
  • Cancer Research

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