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Musculoskeletal
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Non-Trauma
Soft Tissue Tumors
Peripheral Nerve Sheath Tumors
Malignant Peripheral Nerve Sheath Tumor
Malignant Peripheral Nerve Sheath Tumor
Malignant Peripheral Nerve Sheath Tumor
Stephanie A. Bernard, MD; Catherine C. Roberts, MD
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KEY FACTS

  • Imaging

    • Pathology

      • Clinical Issues

        TERMINOLOGY

        • Abbreviations

          • Malignant peripheral nerve sheath tumor (MPNST)

        • Synonyms

          • Not recommended: malignant schwannoma, neurogenic sarcoma, malignant neurilemoma, neurofibrosarcoma

        IMAGING

        • General Features

          • Radiographic Findings

            • CT Findings

              • MR Findings

                • Nuclear Medicine Findings

                  • Ultrasonographic Findings

                    • Angiographic Findings

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Gross Pathologic & Surgical Features

                            • Microscopic Features

                              • Immunohistochemistry

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        Selected References

                                        1. Hassan A et al: Systemic options for malignant peripheral nerve sheath tumors. Curr Treat Options Oncol. 22(4):33, 2021
                                        2. Yun JS et al: Peripheral nerve sheath tumor: differentiation of malignant from benign tumors with conventional and diffusion-weighted MRI. Eur Radiol. 31(3):1548-57, 2021
                                        3. Ahlawat S et al: Current status and recommendations for imaging in neurofibromatosis type 1, neurofibromatosis type 2, and schwannomatosis. Skeletal Radiol. 49(2):199-219, 2020
                                        4. Tovmassian D et al: The role of [18F]FDG-PET/CT in predicting malignant transformation of plexiform neurofibromas in neurofibromatosis-1. Int J Surg Oncol. 2016:6162182, 2016
                                        5. Schaefer IM et al: Malignant peripheral nerve sheath tumor (MPNST) arising in diffuse-type neurofibroma: clinicopathologic characterization in a series of 9 cases. Am J Surg Pathol. 39(9):1234-41, 2015
                                        6. Wasa J et al: MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas. AJR Am J Roentgenol. 194(6):1568-74., 2010
                                        7. Benz MR et al: Quantitative F18-fluorodeoxyglucose positron emission tomography accurately characterizes peripheral nerve sheath tumors as malignant or benign. Cancer. 116(2):451-8, 2009
                                        8. Karabatsou K et al: Potential role of 18fluorodeoxyglucose-positron emission tomography/computed tomography in differentiating benign neurofibroma from malignant peripheral nerve sheath tumor associated with neurofibromatosis 1. Neurosurgery. 65(4 Suppl):160-70, 2009
                                        9. Grobmyer SR et al: Malignant peripheral nerve sheath tumor: molecular pathogenesis and current management considerations. J Surg Oncol. 97(4):340-9, 2008
                                        10. Weiss SW et al: Malignant tumors of the peripheral nerve. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 903-25, 2008
                                        11. Park SK et al: Metastasizing malignant peripheral nerve sheath tumor initially presenting as intracerebral hemorrhage. Case report and review of the literature. Surg Neurol. 68(1):79-84; discussion 84, 2007
                                        12. Kransdorf MJ et al: Neurogenic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Lippincott Williams & Wilkins. 337-8, 2006
                                        13. Lee YJ et al: Malignant peripheral nerve sheath tumor arising from the colon in a newborn: report of a case and review of the literatures. J Pediatr Surg. 41(2):e19-22, 2006
                                        14. Keel SB et al: Orthopaedic implant-related sarcoma: a study of twelve cases. Mod Pathol. 14(10):969-77, 2001
                                        15. Navarro O et al: Malignant peripheral nerve-sheath tumor arising in a previously irradiated neuroblastoma: report of 2 cases and a review of the literature. Pediatr Radiol. 30(3):176-80, 2000
                                        16. Khan RJ et al: Primary intraosseous malignant peripheral nerve sheath tumor: a case report and review of the literature. Pathology. 30(3):237-41, 1998
                                        17. Terry DG et al: Intraosseous malignant peripheral nerve sheath tumor in a patient with neurofibromatosis. Skeletal Radiol. 27(6):346-9, 1998
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Imaging

                                          • Pathology

                                            • Clinical Issues

                                              TERMINOLOGY

                                              • Abbreviations

                                                • Malignant peripheral nerve sheath tumor (MPNST)

                                              • Synonyms

                                                • Not recommended: malignant schwannoma, neurogenic sarcoma, malignant neurilemoma, neurofibrosarcoma

                                              IMAGING

                                              • General Features

                                                • Radiographic Findings

                                                  • CT Findings

                                                    • MR Findings

                                                      • Nuclear Medicine Findings

                                                        • Ultrasonographic Findings

                                                          • Angiographic Findings

                                                            DIFFERENTIAL DIAGNOSIS

                                                              PATHOLOGY

                                                              • General Features

                                                                • Gross Pathologic & Surgical Features

                                                                  • Microscopic Features

                                                                    • Immunohistochemistry

                                                                      CLINICAL ISSUES

                                                                      • Presentation

                                                                        • Demographics

                                                                          • Natural History & Prognosis

                                                                            • Treatment

                                                                              Selected References

                                                                              1. Hassan A et al: Systemic options for malignant peripheral nerve sheath tumors. Curr Treat Options Oncol. 22(4):33, 2021
                                                                              2. Yun JS et al: Peripheral nerve sheath tumor: differentiation of malignant from benign tumors with conventional and diffusion-weighted MRI. Eur Radiol. 31(3):1548-57, 2021
                                                                              3. Ahlawat S et al: Current status and recommendations for imaging in neurofibromatosis type 1, neurofibromatosis type 2, and schwannomatosis. Skeletal Radiol. 49(2):199-219, 2020
                                                                              4. Tovmassian D et al: The role of [18F]FDG-PET/CT in predicting malignant transformation of plexiform neurofibromas in neurofibromatosis-1. Int J Surg Oncol. 2016:6162182, 2016
                                                                              5. Schaefer IM et al: Malignant peripheral nerve sheath tumor (MPNST) arising in diffuse-type neurofibroma: clinicopathologic characterization in a series of 9 cases. Am J Surg Pathol. 39(9):1234-41, 2015
                                                                              6. Wasa J et al: MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas. AJR Am J Roentgenol. 194(6):1568-74., 2010
                                                                              7. Benz MR et al: Quantitative F18-fluorodeoxyglucose positron emission tomography accurately characterizes peripheral nerve sheath tumors as malignant or benign. Cancer. 116(2):451-8, 2009
                                                                              8. Karabatsou K et al: Potential role of 18fluorodeoxyglucose-positron emission tomography/computed tomography in differentiating benign neurofibroma from malignant peripheral nerve sheath tumor associated with neurofibromatosis 1. Neurosurgery. 65(4 Suppl):160-70, 2009
                                                                              9. Grobmyer SR et al: Malignant peripheral nerve sheath tumor: molecular pathogenesis and current management considerations. J Surg Oncol. 97(4):340-9, 2008
                                                                              10. Weiss SW et al: Malignant tumors of the peripheral nerve. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Elsevier. 903-25, 2008
                                                                              11. Park SK et al: Metastasizing malignant peripheral nerve sheath tumor initially presenting as intracerebral hemorrhage. Case report and review of the literature. Surg Neurol. 68(1):79-84; discussion 84, 2007
                                                                              12. Kransdorf MJ et al: Neurogenic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Lippincott Williams & Wilkins. 337-8, 2006
                                                                              13. Lee YJ et al: Malignant peripheral nerve sheath tumor arising from the colon in a newborn: report of a case and review of the literatures. J Pediatr Surg. 41(2):e19-22, 2006
                                                                              14. Keel SB et al: Orthopaedic implant-related sarcoma: a study of twelve cases. Mod Pathol. 14(10):969-77, 2001
                                                                              15. Navarro O et al: Malignant peripheral nerve-sheath tumor arising in a previously irradiated neuroblastoma: report of 2 cases and a review of the literature. Pediatr Radiol. 30(3):176-80, 2000
                                                                              16. Khan RJ et al: Primary intraosseous malignant peripheral nerve sheath tumor: a case report and review of the literature. Pathology. 30(3):237-41, 1998
                                                                              17. Terry DG et al: Intraosseous malignant peripheral nerve sheath tumor in a patient with neurofibromatosis. Skeletal Radiol. 27(6):346-9, 1998