Peripheral nerve tumors
- James M Gilchrist, MD
James M Gilchrist, MD
- Professor and Chair, Department of Neurology
- Southern Illinois University School of Medicine
- John E Donahue, MD
John E Donahue, MD
- Assistant Professor of Pathology and Laboratory Medicine and Neurology
- Rhode Island Hospital, Warren Alpert Medical School of Brown University
- Section Editors
- Jeremy M Shefner, MD, PhD
Jeremy M Shefner, MD, PhD
- Section Editor — Neuromuscular Disease
- Professor and Chair of Neurology, Barrow Neurological Institute
- Professor of Neurology, University of Arizona, Phoenix
- Clinical Professor of Neurology, Creighton University
- Robert Maki, MD, PhD
Robert Maki, MD, PhD
- Section Editor — Bone and Soft Tissue Tumors
- Professor of Medicine
- Monter Cancer Center
- Hofstra-Northwell School of Medicine
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 . 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 . 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 . A careful family history is important in the assessment of an underlying neurogenetic disorder, such as neurofibromatosis.
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- CLINICAL PRESENTATION
- LABORATORY TESTING
- Electrodiagnostic testing
- APPROACH TO TREATMENT
- BENIGN NON-NEOPLASTIC NERVE TUMORS
- Ganglion cyst
- Intraneural heterotopic ossification
- Sarcoid granuloma
- Inflammatory pseudotumor of nerve
- Hypertrophic neuropathy
- BENIGN NEOPLASMS OF NON-NEURAL SHEATH ORIGIN
- Desmoid tumor
- BENIGN NERVE SHEATH NEOPLASMS
- Hybrid nerve sheath tumors
- Dermal nerve sheath myxoma
- MALIGNANT PERIPHERAL NERVE SHEATH TUMORS
- Treatment of MPNSTs
- MALIGNANT NON-NEURAL SHEATH TUMORS
- INFORMATION FOR PATIENTS