RESEARCH ARTICLE


Polyradiculopathies from Schwannomatosis



Yuxia Jia1, James A. Kraus2, Hasini Reddy2, Michael Groff 3, Eric T Wong*, 4
1 Division of Gerontology, Department of Medicine, Beth Israel Deaconess Medical Center
2 Department of Pathology, Beth Israel Deaconess Medical Center
3 Division of Neurosurgery, Beth Israel Deaconess Medical Center
4 Brain Tumor Center & Neuro-Oncology Unit, Department of Neurology, Beth Israel Deaconess Medical Center, USA


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© Jia et al; Licensee Bentham Open

open-access license: This is an open access article licensed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.

* Address correspondence to this author at the Brain Tumor Center & Neuro-Oncology Unit, Department of Neurology, Beth Israel Deaconess Medical Center, 330 Brookline Avenue, Boston, MA 02215, USA; Tel: (617) 667-1665; Fax: (617) 667-1664; E-mail: ewong@bidmc.harvard.edu


Abstract

We describe a case of schwannomatosis presenting as radicular pain and numbness in multiple radicular nerve distributions. There were multiple peripheral nerve tumors detected by magnetic resonance imaging (MRI) at the left vestibular nerve, cauda equina, right radial nerve, thoracic paraspinal nerve, and brachial plexi. Several resected tumors have features of schwannomas, including hypercellular Antoni A areas, hypocellular Antoni B areas, Verocay bodies, and hyalinized blood vessels. The specimens are also positive for immunohistochemical staining for INI1 with diffuse nuclear staining. The findings are consistent with sporadic form of schwannomatosis. This case highlights the importance of using MRI and INI1 immunohistochemistry to differentiate familial schwannomatosis, neurofibromatosis 2 (NF2)-associated schwannomatosis, and sporadic schwannomatosis.

Keywords: Schwannomatosis, polyradiculopathies, INI1 immunohistochemistry.