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

Purpose: To report the onset of malignant peripheral nerve sheath tumor of the orbit 8 years after irradiation in a patient with neurofibromatosis typ e-1. Methods: Case report of a young man with neurofibromatosis type-1 who recei ved irradiation for presumed bilateral optic nerve and chiasmal gliomas and in whom a malignant peripheral nerve sheath tumor later developed. Exenter ation with extirpation of the entire contents of the orbit was performed 6 times. Results: Complete recurrence of the tumor occurred after each surgical proc edure until the patient died of malignancy. Conclusions: Our case underscores the risk of irradiation, especially in ch ildren with neurofibromatosis type-1, and emphasizes that radiotherapy shou ld never be given as an empirical therapy. The authors believe that irradia tion and neurofibromatosis type-1 may, in combination, pose a significant r isk for the development of malignancies. Clear-cut indications and a precis e tissue diagnosis are desirable before the initiation of radiotherapy, par ticularly in the pediatric population. We recommend that if irradiation is necessary in persons with neurofibromatosis type-1, regular follow-up is im perative. In view of the hostile nature of malignant peripheral nerve sheat h tumor, early aggressive treatment appears to be the only viable alternati ve at present.