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Peripheral nerve tumors

James M Gilchrist, MD
John E Donahue, MD
Section Editors
Jeremy M Shefner, MD, PhD
Robert Maki, MD, PhD
Deputy Editor
April F Eichler, MD, MPH


Peripheral nerve tumors are uncommon and many neurologists will see only a handful in their careers, unless they have a practice enriched in patients with neurofibromatosis.

This topic review will focus on various benign and malignant neoplasms and benign non-neoplastic tumors of the peripheral nerve. The neurofibromatoses are discussed separately. (See "Neurofibromatosis type 1 (NF1): Pathogenesis, clinical features, and diagnosis" and "Neurofibromatosis type 2" and "Schwannomatosis".)

Nerve sheath tumors affecting the spinal cord are reviewed elsewhere. (See "Intradural nerve sheath tumors".)


Symptoms and signs of peripheral nerve tumors are caused by direct nerve invasion, involvement of surrounding tissues, or mass effect [1]. There are no specific clinical presentations unique or even especially suggestive of a particular nerve tumor, with the exception of neurofibromatosis type 1 and neurofibromatosis type 2. (See "Neurofibromatosis type 1 (NF1): Pathogenesis, clinical features, and diagnosis" and "Neurofibromatosis type 2".)

Patients present for evaluation of peripheral nerve tumors because of a soft tissue mass, pain, or focal neurologic findings, approximately in that order of frequency [2]. The duration and progression of symptoms or signs is important as most benign tumors have a longer duration and a slow rate of progression, while malignant tumors tend to progress rapidly in size, amount of pain, and neurologic deficit [2-4]. Any mass changing its clinical character should be taken seriously, as that is one of the few clinical clues to its potentially malignant nature. In particular, rapidly expanding soft tissue masses are very suspicious for the presence of a malignant peripheral nerve sheath tumor and should be evaluated promptly [3]. A careful family history is important in the assessment of an underlying neurogenetic disorder, such as neurofibromatosis.

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Literature review current through: Nov 2017. | This topic last updated: Jul 03, 2017.
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