1. Multiple Recurrences in Malignant Peripheral Nerve Sheath Tumor of the Orbit
- Author
-
Gordon K. Klintworth, Christopher M. DeBacker, William J. Lipham, Hatem A. Tawfik, Gregg S. Gayre, and Jonathan J. Dutton
- Subjects
Male ,medicine.medical_specialty ,Neoplasms, Radiation-Induced ,Neurofibromatosis 1 ,Eye disease ,medicine.medical_treatment ,Malignant peripheral nerve sheath tumor ,Malignancy ,Nerve Sheath Neoplasms ,Fatal Outcome ,Humans ,Medicine ,Neurofibromatosis ,Orbit Evisceration ,business.industry ,Optic Nerve Neoplasms ,Glioma ,General Medicine ,medicine.disease ,Magnetic Resonance Imaging ,Surgery ,Radiation therapy ,Ophthalmology ,medicine.anatomical_structure ,Child, Preschool ,Optic Chiasm ,Optic nerve ,Orbital Neoplasms ,Neoplasm Recurrence, Local ,business ,Complication ,Orbit (anatomy) - Abstract
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.
- Published
- 2001