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Acta Neurochirurgica
Published in: Acta Neurochirurgica 5/2013

01-05-2013 | Clinical Article - Spine

Prognostic value and management of spinal tumors in neurofibromatosis type 2 patients

Authors: Rabih Aboukais, Marc Baroncini, Fahed Zairi, Nicolas-Xavier Bonne, Stéphane Schapira, Christophe Vincent, Jean-Paul Lejeune

Published in: Acta Neurochirurgica | Issue 5/2013

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Abstract

Purpose

Neurofibromatosis type 2 (NF2) is a well-studied disease. Although spinal tumors are frequent, many issues concerning their prognosis and management still have to be clarified. The authors studied the clinical impact and radiological progression of spinal tumors in patients with NF2 to determine their prognostic value.

Methods

A total of 80 NF2 patients were followed in the Lille University Hospital between 1987 and 2011. Clinical, radiological and genetic data were retrospectively recorded and analyzed. Patients underwent annual cranial and spinal MRI. Both the location and size of each tumor were reported. The diagnosis of NF2 was confirmed either because the patient met the Manchester criteria or by the presence of genetic mutation.

Results

The mean follow-up period was 8.8 years (range 1 to 24 years; SD: ±0.8), and the mean age at diagnosis was 27.2 years (range 6 to 73 years; SD: ±1.7). Among all patients, 48 harbored spinal tumors. Twenty of them were symptomatic, and 21 were operated on. Patients with spinal tumors had a lower age at diagnosis (p = 0.02), a higher number of intracranial meningiomas (p = 0.028) and schwannomas (p = 0.03), and more nonsense and frameshift mutations (p = 0.04).

Conclusion

Spinal tumors are common in NF2, and all patients should be regularly monitored by spinal MRI. The presence of spinal tumors seems to be a factor indicating poor prognosis. Clinical and radiological monitoring of spinal tumors could lead to early treatment both when clinical symptoms are present and in case of proven radiological evolution, maintaining a favorable functional prognosis as long as possible.
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Metadata
Title
Prognostic value and management of spinal tumors in neurofibromatosis type 2 patients
Authors
Rabih Aboukais
Marc Baroncini
Fahed Zairi
Nicolas-Xavier Bonne
Stéphane Schapira
Christophe Vincent
Jean-Paul Lejeune
Publication date
01-05-2013
Publisher
Springer Vienna
Published in
Acta Neurochirurgica / Issue 5/2013
Print ISSN: 0001-6268
Electronic ISSN: 0942-0940
DOI
https://doi.org/10.1007/s00701-012-1590-z

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