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Malignant Peripheral Nerve Sheath Tumor
Catherine C. Roberts, MD
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KEY FACTS

  • Imaging

    • Pathology

      • Clinical Issues

        TERMINOLOGY

        • Abbreviations

          • Malignant peripheral nerve sheath tumor (MPNST)
        • Synonyms

          • Malignant schwannoma, neurogenic sarcoma, malignant neurilemmoma, 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

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      Selected References

                                      1. 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. ePub, 2015
                                      2. Benz MR et al: Quantitative F18-fluorodeoxyglucose positron emission tomography accurately characterizes peripheral nerve sheath tumors as malignant or benign. Cancer. 116(2):451-458, 2009
                                      3. Wasa J, Nishida Y, Tsukushi S, et al. MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas. AJR Am J Roentgenol. 194(6):1568-74., 2010
                                      4. 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
                                      5. Grobmyer SR et al: Malignant peripheral nerve sheath tumor: molecular pathogenesis and current management considerations. J Surg Oncol. 97(4):340-9, 2008
                                      6. Weiss SW et al: Malignant tumors of the peripheral nerve. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Philadelphia: Elsevier. 903-25, 2008
                                      7. 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
                                      8. Kransdorf MJ et al: Neurogenic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Philadelphia: Lippincott Williams & Wilkins. 337-8, 2006
                                      9. 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
                                      10. Keel SB et al: Orthopaedic implant-related sarcoma: a study of twelve cases. Mod Pathol. 14(10):969-77, 2001
                                      11. 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
                                      12. 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
                                      13. 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

                                              • Malignant schwannoma, neurogenic sarcoma, malignant neurilemmoma, 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

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          Selected References

                                                                          1. 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. ePub, 2015
                                                                          2. Benz MR et al: Quantitative F18-fluorodeoxyglucose positron emission tomography accurately characterizes peripheral nerve sheath tumors as malignant or benign. Cancer. 116(2):451-458, 2009
                                                                          3. Wasa J, Nishida Y, Tsukushi S, et al. MRI features in the differentiation of malignant peripheral nerve sheath tumors and neurofibromas. AJR Am J Roentgenol. 194(6):1568-74., 2010
                                                                          4. 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
                                                                          5. Grobmyer SR et al: Malignant peripheral nerve sheath tumor: molecular pathogenesis and current management considerations. J Surg Oncol. 97(4):340-9, 2008
                                                                          6. Weiss SW et al: Malignant tumors of the peripheral nerve. In Weiss SW et al: Enzinger and Weiss' Soft Tissue Tumors. 5th ed. Philadelphia: Elsevier. 903-25, 2008
                                                                          7. 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
                                                                          8. Kransdorf MJ et al: Neurogenic tumors. In Kransdorf MJ et al: Imaging of Soft Tissue Tumors. 2nd ed. Philadelphia: Lippincott Williams & Wilkins. 337-8, 2006
                                                                          9. 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
                                                                          10. Keel SB et al: Orthopaedic implant-related sarcoma: a study of twelve cases. Mod Pathol. 14(10):969-77, 2001
                                                                          11. 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
                                                                          12. 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
                                                                          13. Terry DG et al: Intraosseous malignant peripheral nerve sheath tumor in a patient with neurofibromatosis. Skeletal Radiol. 27(6):346-9, 1998