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J Neurol Surg A Cent Eur Neurosurg 2018; 79(05): 408-415
DOI: 10.1055/s-0038-1655548
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Sporadic Schwannomatosis: A Systematic Review Following the 2005 Consensus Statement

Gregoire Chick
1   Department of Hand Surgery and Peripheral Nerve Surgery, Hôpital de la Tour, Geneva, Switzerland
2   Department of Orthopaedic Surgery and Traumatology, Ghent University Hospital, Gent, Belgium
,
Jan Victor
2   Department of Orthopaedic Surgery and Traumatology, Ghent University Hospital, Gent, Belgium
,
Thibault Poujade
1   Department of Hand Surgery and Peripheral Nerve Surgery, Hôpital de la Tour, Geneva, Switzerland
,
Nadine Hollevoet
2   Department of Orthopaedic Surgery and Traumatology, Ghent University Hospital, Gent, Belgium
› Author Affiliations
Further Information

Publication History

08 August 2017

29 March 2018

Publication Date:
14 June 2018 (online)

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Abstract

Aims To identify the frequency of reports of sporadic schwannomatosis, the types of patients affected, and the nerves affected.

Patients and Methods We identified all case reports and case series that reported on patients with sporadic schwannomatosis according to established criteria.

Results The initial search yielded 1,597 studies, of which 15 were included. A total of 38 of 55 individuals met the inclusion criteria. The mean age of the patients was 48 years; 41% were male. Thirty-three patients had peripheral nerve tumors, and 17 had spinal tumors. Twelve had tumors in both locations. Tumor distribution was unilateral in 25 of 30 cases (83.3%) and segmental (limited to one limb or five or fewer contiguous segments of the spine) in 28 of 38 cases (73.7%).

Conclusion This systematic review quantified the number of individuals who meet the criteria for sporadic schwannomatosis and better described this population to facilitate the classification of neurofibromatosis in regard to the 2005 consensus statement. Unilateral or segmental distribution of nerve tumors are key aspects when dealing with multiple noncutaneous schwannomas without involvement of the vestibular nerve.

Keywords

Schwannomas - peripheral nerve tumors - schwannomatosis - neurofibromatosis
 

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