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Spine
Diagnosis
Peripheral Nerve and Plexus
Plexus and Peripheral Nerve Lesions
Peripheral Nerve Sheath Tumor
Peripheral Nerve Sheath Tumor
Kevin R. Moore, MD; Jeffrey S. Ross, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Definitions

                • Grouping of benign and malignant primary tumors of peripheral nerves
                • Malignant peripheral nerve sheath tumor (MPNST) replaces earlier terms (e.g., malignant schwannoma, neurofibrosarcoma, neurogenic sarcoma)

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Imaging Recommendations

                      • Ultrasonographic Findings

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              • Reporting Tips

                                                Selected References

                                                1. Ahlawat S et al: Imaging biomarkers for malignant peripheral nerve sheath tumors in neurofibromatosis type 1. Neurology. 93(11):e1076-84, 2019
                                                2. Assadi M et al: PET imaging of peripheral nerve tumors. PET Clin. 14(1):81-9, 2019
                                                3. Well L et al: Differentiation of peripheral nerve sheath tumors in patients with neurofibromatosis type 1 using diffusion-weighted magnetic resonance imaging. Neuro Oncol. 21(4):508-16, 2019
                                                4. James AW et al: Malignant peripheral nerve sheath tumor. Surg Oncol Clin N Am. 25(4):789-802, 2016
                                                5. Soldatos T et al: Advanced MR imaging of peripheral nerve sheath tumors including diffusion imaging. Semin Musculoskelet Radiol. 19(2):179-90, 2015
                                                6. Wang T et al: Malignant peripheral nerve sheath tumor (MPNST) in the spine: a retrospective analysis of clinical and molecular prognostic factors. J Neurooncol. 122(2):349-55, 2015
                                                7. Mullins BT et al: Malignant peripheral nerve sheath tumors of the head and neck: a case series and literature review. Case Rep Otolaryngol. 2014:368920, 2014
                                                8. Gan HK et al: Neurolymphomatosis: diagnosis, management, and outcomes in patients treated with rituximab. Neuro Oncol. 12(2):212-5, 2010
                                                9. Levi AD et al: The surgical management of symptomatic peripheral nerve sheath tumors. Neurosurgery. 66(4):833-40, 2010
                                                10. Arun D et al: Recent advances in neurofibromatosis type 1. Curr Opin Neurol. 17(2):101-5, 2004
                                                11. Beaulieu S et al: Positron emission tomography of schwannomas: emphasizing its potential in preoperative planning. AJR Am J Roentgenol. 182(4):971-4, 2004
                                                12. Beekman R et al: Bilateral intraneural perineurioma presenting as ulnar neuropathy at the elbow. Muscle Nerve. 30(2):239-43, 2004
                                                13. Dellon AL et al: Painful neuroma of the posterior cutaneous nerve of the forearm after surgery for lateral humeral epicondylitis. J Hand Surg [Am]. 29(3):387-90, 2004
                                                14. Forthman CL et al: Nerve tumors of the hand and upper extremity. Hand Clin. 20(3):233-42, v, 2004
                                                15. He Y et al: The application of magnetic resonance imaging-guided fine-needle aspiration cytology in the diagnosis of deep lesions in the head and neck. J Oral Maxillofac Surg. 62(8):953-8, 2004
                                                16. Hochman MG et al: Nerves in a pinch: imaging of nerve compression syndromes. Radiol Clin North Am. 42(1):221-45, 2004
                                                17. Huang JH et al: Surgical management of brachial plexus region tumors. Surg Neurol. 61(4):372-8, 2004
                                                18. Kim DH et al: Management and outcomes in 318 operative common peroneal nerve lesions at the Louisiana State University Health Sciences Center. Neurosurgery. 54(6):1421-8; discussion 1428-9, 2004
                                                19. Lin V et al: Is a plexiform neurofibroma pathognomonic of neurofibromatosis type I? Laryngoscope. 114(8):1410-4, 2004
                                                20. Robertson TC et al: Isolated plexiform neurofibroma: treatment with three-dimensional conformal radiotherapy. Laryngoscope. 114(7):1139-42, 2004
                                                21. Rosai J: Surgical Pathology, vol 2. 9th ed. Mosby. 2629-62, 2004
                                                22. Shapiro SL: Endoscopic decompression of the intermetatarsal nerve for Morton's neuroma. Foot Ankle Clin. 9(2):297-304, 2004
                                                23. Skubitz KM et al: Characterization of sarcomas by means of gene expression. J Lab Clin Med. 144(2):78-91, 2004
                                                24. Stamatis ED et al: Interdigital neuromas: current state of the art--surgical. Foot Ankle Clin. 9(2):287-96, 2004
                                                25. Stemmer-Rachamimov AO et al: Comparative pathology of nerve sheath tumors in mouse models and humans. Cancer Res. 64(10):3718-24, 2004
                                                26. Velagaleti GV et al: Malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation (malignant triton tumor) with balanced t(7;9)(q11.2;p24) and unbalanced translocation der(16)t(1;16)(q23;q13). Cancer Genet Cytogenet. 149(1):23-7, 2004
                                                27. Mohan H et al: Peripheral nerve sheath tumours--a short series with some uncommon variants. Indian J Pathol Microbiol. 46(2):204-6, 2003
                                                28. Weiss SW et al: Soft Tissue Tumors. 4th ed. Mosby. 1111-263, 2001
                                                Related Anatomy
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                                                Related Differential Diagnoses
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                                                References
                                                Tables

                                                Tables

                                                KEY FACTS

                                                • Terminology

                                                  • Imaging

                                                    • Top Differential Diagnoses

                                                      • Pathology

                                                        • Clinical Issues

                                                          • Diagnostic Checklist

                                                            TERMINOLOGY

                                                            • Definitions

                                                              • Grouping of benign and malignant primary tumors of peripheral nerves
                                                              • Malignant peripheral nerve sheath tumor (MPNST) replaces earlier terms (e.g., malignant schwannoma, neurofibrosarcoma, neurogenic sarcoma)

                                                            IMAGING

                                                            • General Features

                                                              • CT Findings

                                                                • MR Findings

                                                                  • Imaging Recommendations

                                                                    • Ultrasonographic Findings

                                                                      DIFFERENTIAL DIAGNOSIS

                                                                        PATHOLOGY

                                                                        • General Features

                                                                          • Staging, Grading, & Classification

                                                                            • Gross Pathologic & Surgical Features

                                                                              • Microscopic Features

                                                                                CLINICAL ISSUES

                                                                                • Presentation

                                                                                  • Demographics

                                                                                    • Natural History & Prognosis

                                                                                      • Treatment

                                                                                        DIAGNOSTIC CHECKLIST

                                                                                        • Consider

                                                                                          • Image Interpretation Pearls

                                                                                            • Reporting Tips

                                                                                              Selected References

                                                                                              1. Ahlawat S et al: Imaging biomarkers for malignant peripheral nerve sheath tumors in neurofibromatosis type 1. Neurology. 93(11):e1076-84, 2019
                                                                                              2. Assadi M et al: PET imaging of peripheral nerve tumors. PET Clin. 14(1):81-9, 2019
                                                                                              3. Well L et al: Differentiation of peripheral nerve sheath tumors in patients with neurofibromatosis type 1 using diffusion-weighted magnetic resonance imaging. Neuro Oncol. 21(4):508-16, 2019
                                                                                              4. James AW et al: Malignant peripheral nerve sheath tumor. Surg Oncol Clin N Am. 25(4):789-802, 2016
                                                                                              5. Soldatos T et al: Advanced MR imaging of peripheral nerve sheath tumors including diffusion imaging. Semin Musculoskelet Radiol. 19(2):179-90, 2015
                                                                                              6. Wang T et al: Malignant peripheral nerve sheath tumor (MPNST) in the spine: a retrospective analysis of clinical and molecular prognostic factors. J Neurooncol. 122(2):349-55, 2015
                                                                                              7. Mullins BT et al: Malignant peripheral nerve sheath tumors of the head and neck: a case series and literature review. Case Rep Otolaryngol. 2014:368920, 2014
                                                                                              8. Gan HK et al: Neurolymphomatosis: diagnosis, management, and outcomes in patients treated with rituximab. Neuro Oncol. 12(2):212-5, 2010
                                                                                              9. Levi AD et al: The surgical management of symptomatic peripheral nerve sheath tumors. Neurosurgery. 66(4):833-40, 2010
                                                                                              10. Arun D et al: Recent advances in neurofibromatosis type 1. Curr Opin Neurol. 17(2):101-5, 2004
                                                                                              11. Beaulieu S et al: Positron emission tomography of schwannomas: emphasizing its potential in preoperative planning. AJR Am J Roentgenol. 182(4):971-4, 2004
                                                                                              12. Beekman R et al: Bilateral intraneural perineurioma presenting as ulnar neuropathy at the elbow. Muscle Nerve. 30(2):239-43, 2004
                                                                                              13. Dellon AL et al: Painful neuroma of the posterior cutaneous nerve of the forearm after surgery for lateral humeral epicondylitis. J Hand Surg [Am]. 29(3):387-90, 2004
                                                                                              14. Forthman CL et al: Nerve tumors of the hand and upper extremity. Hand Clin. 20(3):233-42, v, 2004
                                                                                              15. He Y et al: The application of magnetic resonance imaging-guided fine-needle aspiration cytology in the diagnosis of deep lesions in the head and neck. J Oral Maxillofac Surg. 62(8):953-8, 2004
                                                                                              16. Hochman MG et al: Nerves in a pinch: imaging of nerve compression syndromes. Radiol Clin North Am. 42(1):221-45, 2004
                                                                                              17. Huang JH et al: Surgical management of brachial plexus region tumors. Surg Neurol. 61(4):372-8, 2004
                                                                                              18. Kim DH et al: Management and outcomes in 318 operative common peroneal nerve lesions at the Louisiana State University Health Sciences Center. Neurosurgery. 54(6):1421-8; discussion 1428-9, 2004
                                                                                              19. Lin V et al: Is a plexiform neurofibroma pathognomonic of neurofibromatosis type I? Laryngoscope. 114(8):1410-4, 2004
                                                                                              20. Robertson TC et al: Isolated plexiform neurofibroma: treatment with three-dimensional conformal radiotherapy. Laryngoscope. 114(7):1139-42, 2004
                                                                                              21. Rosai J: Surgical Pathology, vol 2. 9th ed. Mosby. 2629-62, 2004
                                                                                              22. Shapiro SL: Endoscopic decompression of the intermetatarsal nerve for Morton's neuroma. Foot Ankle Clin. 9(2):297-304, 2004
                                                                                              23. Skubitz KM et al: Characterization of sarcomas by means of gene expression. J Lab Clin Med. 144(2):78-91, 2004
                                                                                              24. Stamatis ED et al: Interdigital neuromas: current state of the art--surgical. Foot Ankle Clin. 9(2):287-96, 2004
                                                                                              25. Stemmer-Rachamimov AO et al: Comparative pathology of nerve sheath tumors in mouse models and humans. Cancer Res. 64(10):3718-24, 2004
                                                                                              26. Velagaleti GV et al: Malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation (malignant triton tumor) with balanced t(7;9)(q11.2;p24) and unbalanced translocation der(16)t(1;16)(q23;q13). Cancer Genet Cytogenet. 149(1):23-7, 2004
                                                                                              27. Mohan H et al: Peripheral nerve sheath tumours--a short series with some uncommon variants. Indian J Pathol Microbiol. 46(2):204-6, 2003
                                                                                              28. Weiss SW et al: Soft Tissue Tumors. 4th ed. Mosby. 1111-263, 2001