Multiple recurrences in malignant peripheral nerve sheath tumor of the orbit: a case report and a review of the literature.


Journal Article (Review)

PURPOSE: To report the onset of malignant peripheral nerve sheath tumor of the orbit 8 years after irradiation in a patient with neurofibromatosis type-1. METHODS: Case report of a young man with neurofibromatosis type-1 who received irradiation for presumed bilateral optic nerve and chiasmal gliomas and in whom a malignant peripheral nerve sheath tumor later developed. Exenteration with extirpation of the entire contents of the orbit was performed 6 times. RESULTS: Complete recurrence of the tumor occurred after each surgical procedure until the patient died of malignancy. CONCLUSIONS: Our case underscores the risk of irradiation, especially in children with neurofibromatosis type-1, and emphasizes that radiotherapy should never be given as an empirical therapy. The authors believe that irradiation and neurofibromatosis type-1 may, in combination, pose a significant risk for the development of malignancies. Clear-cut indications and a precise tissue diagnosis are desirable before the initiation of radiotherapy, particularly in the pediatric population. We recommend that if irradiation is necessary in persons with neurofibromatosis type-1, regular follow-up is imperative. In view of the hostile nature of malignant peripheral nerve sheath tumor, early aggressive treatment appears to be the only viable alternative at present.

Full Text

Cited Authors

  • Dutton, JJ; Tawfik, HA; DeBacker, CM; Lipham, WJ; Gayre, GS; Klintworth, GK

Published Date

  • July 2001

Published In

Volume / Issue

  • 17 / 4

Start / End Page

  • 293 - 299

PubMed ID

  • 11476181

Pubmed Central ID

  • 11476181

International Standard Serial Number (ISSN)

  • 0740-9303

Digital Object Identifier (DOI)

  • 10.1097/00002341-200107000-00010


  • eng

Conference Location

  • United States