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Schwannomatosis, sporadic schwannomatosis, and familial schwannomatosis: a surgical series with long-term follow-up.
MedLine Citation:
PMID:  20932094     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Object The aim of this study was to provide disease-specific information about schwannomatosis in its different forms and to present 2 particular cases of malignant schwannomas in the context of familial schwannomatosis (FS). Methods The authors analyzed patients with pathologically defined schwannomas and identified those with varied forms of schwannomatosis. Each case was retrospectively analyzed for patient sex and age, number of operations and tumors excised, symptoms, location and size of tumors, extent of resection, nerve function pre- and postoperatively, complications, other nonsurgically treated tumors, malignancy, results of brain MR imaging, and follow-up data. Results One hundred fifty-eight patients underwent the excision of 216 schwannomas. One hundred forty-two patients presented with solitary schwannomas, 2 had neurofibromatosis Type 2 (NF2), and 14 presented with schwannomatosis. The average follow-up was 52 months. Six individuals had sporadic schwannomatosis, whereas 8 had the familial form of the disease. These 14 patients had an average age of 28.3 years at the time of disease onset (median 27.5 years) and 35.4 years at the time of the first operation (median 37 years) Thirteen of the 14 patients with schwannomatosis experienced pain as the first symptom. Eight (57%) of the 14 patients presented with at least 1 tumor in the spinal canal or attached to the spinal nerve roots. Malignant schwannomas developed in 2 patients from the same family during the follow-up. Conclusions Patients suffering from schwannomatosis tend to be younger than those presenting with solitary schwannomas. Therefore, individuals presenting at a young age with multiple schwannomas but not meeting the criteria for NF2 should prompt the physician to suspect schwannomatosis. Patients with schwannomatosis who report pain should be exhaustively examined. The spine is affected in the majority of patients, and MR imaging of the spine should be part of the routine evaluation. Rapid enlargement of schwannomas in the context of FS should raise suspicion of malignant transformation.
Authors:
Augusto Gonzalvo; Adam Fowler; Raymond John Cook; Nicholas Scott Little; Helen Wheeler; Kerrie L McDonald; Michael Thomas Biggs
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Publication Detail:
Type:  Journal Article     Date:  2010-10-08
Journal Detail:
Title:  Journal of neurosurgery     Volume:  114     ISSN:  1933-0693     ISO Abbreviation:  J. Neurosurg.     Publication Date:  2011 Mar 
Date Detail:
Created Date:  2011-03-02     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0253357     Medline TA:  J Neurosurg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  756-62     Citation Subset:  AIM; IM    
Affiliation:
Department of Neurosurgery, Royal North Shore Hospital and North Shore Private Hospital; and.
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