Surgery of spinal nerve sheath tumors with special reference to neurofibromatosis.
OBJECTIVE
We conducted a retrospective study of 87 patients with spinal nerve sheath tumors to determine the overall clinical outcome and specific features in 20 patients affected with neurofibromatosis Types 1 and 2 (NF-1 and NF-2, respectively).


METHODS
Case records, operation notes, outpatient files, and radiological examinations were analyzed for all patients treated between September 1977 and August 1994. Additional follow-up data were obtained using outpatient examinations, questionnaires, and telephone calls.


RESULTS
During the study period, 128 spinal neuromas (i.e., schwannomas) and 6 neurofibromas in 87 patients were treated. Fifty-seven neuromas were associated with NF-2 in 17 patients and six neurofibromas with NF-1 in 3 patients. Patients with NF-2 and symptomatic neuromas presented with more severe neurological deficits compared to patients without NF-2. Eighty-six percent of the neuromas were removed completely. On average, most preoperative deficits or symptoms improved in patients without NF-2, whereas neurological symptoms remained unchanged in patients with NF-2. Multiple regression analysis revealed that partial removal, surgery of a recurrent tumor, NF-2, and old age predisposed for tumor recurrence. No increased risk of recurrence was observed for patients with NF-1. For patients without NF-2, we observed overall recurrence rates of 10.7% after 5 years and 28.2% after 10 and 15 years, respectively, as determined by Kaplan-Meier analysis. For NF-2, the recurrence rate at 5 years was 39.2%, and all tumors had recurred by 9 years.


CONCLUSIONS
Spinal nerve sheath tumors carry an excellent prognosis in patients with NF-1 and in patients without neurofibromatosis. Symptomatic neuromas occurring in association with NF-2 present with more severe neurological deficits, demonstrate little postoperative improvement, and have a very high recurrence rate.
Klekamp
J
J
Medical School of Hannover, Neurosurgical Clinic, Nordstadt Hospital, Germany.
Samii
M
M
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D005260:Female; D005544:Forecasting; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D009442:Neurilemmoma / Q000175:diagnosis / Q000601:surgery*; D009456:Neurofibromatosis 1 / Q000175:diagnosis / Q000601:surgery*; D016518:Neurofibromatosis 2 / Q000175:diagnosis / Q000601:surgery*; D010524:Peripheral Nervous System Neoplasms / Q000175:diagnosis / Q000601:surgery*; D012189:Retrospective Studies; D012307:Risk Factors; D013127:Spinal Nerves / Q000473:pathology / Q000601:surgery*; D016896:Treatment Outcome