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Intradural nerve sheath tumors

Ilya Laufer, MD
Mark Bilsky, MD
Section Editors
Robert Maki, MD, PhD
Jay S Loeffler, MD
Patrick Y Wen, MD
Alberto S Pappo, MD
Deputy Editor
April F Eichler, MD, MPH


Spinal tumors are classified as extradural, intradural and extramedullary, or intradural intramedullary based upon their anatomic location. Intramedullary tumors involve the spinal cord, while extramedullary tumors arise outside of the spinal cord. Nerve sheath tumors (NSTs) constitute about 25 percent of tumors arising in the intradural extramedullary space [1,2].

Although the majority of NSTs are confined to the intradural extramedullary space, some of these tumors extend into either the extradural compartment or the spinal cord. In addition, occasional spinal NSTs are confined to either the extradural or intramedullary spaces [3].

NSTs are derived from the Schwann cells and perineurial cells of the peripheral nervous system. Approximately 65 percent of intradural nerve sheath tumors are schwannomas, and most of the remainder are neurofibromas [4]. Malignant NSTs are rare, constituting about 5 percent of such tumors.

The clinical presentation, diagnosis, and treatment of NSTs will be reviewed here. Peripheral extradural nerve sheath tumors, intramedullary spinal tumors, and meningiomas of the spinal cord are discussed separately. (See "Spinal cord tumors", section on 'Intramedullary tumors' and "Spinal cord tumors", section on 'Meningioma' and "Clinical presentation, histopathology, diagnostic evaluation, and staging of soft tissue sarcoma", section on 'Introduction'.)


Intradural, extramedullary nerve sheath tumors (NSTs) may be either sporadic or associated with one of three inherited disorders: neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and schwannomatosis.

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Literature review current through: Nov 2017. | This topic last updated: Mar 29, 2016.
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