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Spine
Diagnosis
Neoplasms, Cysts, and Other Masses
Neoplasms
Intradural Extramedullary
Malignant Nerve Sheath Tumors
Malignant Nerve Sheath Tumors
Jeffrey S. Ross, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Malignant peripheral nerve sheath tumor (MPNST)

            • Synonyms

              • Neurofibrosarcoma
              • Malignant schwannoma
              • Malignant neurilemmoma
              • Neurogenic sarcoma

            • Definitions

              • Soft tissue sarcoma that arises from or differentiates toward cells of peripheral nerve sheath

            IMAGING

            • General Features

              • Radiographic Findings

                • CT Findings

                  • MR Findings

                    • Angiographic Findings

                      • Nuclear Medicine Findings

                        • Imaging Recommendations

                          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

                                              Selected References

                                              1. Widemann BC et al: Biology and management of undifferentiated pleomorphic sarcoma, myxofibrosarcoma, and malignant peripheral nerve sheath tumors: state of the art and perspectives. J Clin Oncol. 36(2):160-7, 2018
                                              2. Kolberg M et al: Survival meta-analyses for >1800 malignant peripheral nerve sheath tumor patients with and without neurofibromatosis type 1. Neuro Oncol. 15(2):135-47, 2013
                                              3. 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
                                              4. 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
                                              5. Ferner RE et al: [18F.] 2-fluoro-2-deoxy-D-glucose positron emission tomography (FDG PET) as a diagnostic tool for neurofibromatosis 1 (NF1) associated malignant peripheral nerve sheath tumours (MPNSTs): a long-term clinical study. Ann Oncol. 19(2):390-4, 2008
                                              6. Gupta G et al: Malignant peripheral nerve sheath tumors. Neurosurg Clin N Am. 19(4):533-43, v, 2008
                                              7. Amin A et al: Radiotherapy-induced malignant peripheral nerve sheath tumor of the cauda equina. Spine (Phila Pa 1976). 29(21):E506-9, 2004
                                              8. Baehring JM et al: Malignant peripheral nerve sheath tumor: the clinical spectrum and outcome of treatment. Neurology. 61(5):696-8, 2003
                                              9. Kluwe L et al: Constitutional NF1 mutations in neurofibromatosis 1 patients with malignant peripheral nerve sheath tumors. Hum Mutat. 22(5):420, 2003
                                              10. Su W et al: Malignant peripheral nerve sheath tumor cell invasion is facilitated by Src and aberrant CD44 expression. Glia. 42(4):350-8, 2003
                                              11. Asavamongkolkul A et al: Malignant peripheral nerve sheath tumor with neurofibromatosis type 1: a 2-case report and review of the literature. J Med Assoc Thai. 84(2):285-93, 2001
                                              12. Stark AM et al: Malignant peripheral nerve sheath tumours--report of 8 cases and review of the literature. Acta Neurochir (Wien). 143(4):357-63; discussion 363-4, 2001
                                              13. Weiss SW et al: Enzinger and Weiss's soft tissue tumors. 4th ed. St. Louis: CV Mosby. 1209-63, 2001
                                              14. Ferner RE et al: Evaluation of (18)fluorodeoxyglucose positron emission tomography ((18)FDG PET) in the detection of malignant peripheral nerve sheath tumours arising from within plexiform neurofibromas in neurofibromatosis 1. J Neurol Neurosurg Psychiatry. 68(3):353-7, 2000
                                              15. Woodruff JM: Pathology of tumors of the peripheral nerve sheath in type 1 neurofibromatosis. Am J Med Genet. 89(1):23-30, 1999
                                              16. Sangüeza OP et al: Neoplasms with neural differentiation: a review. Part II: Malignant neoplasms. Am J Dermatopathol. 20(1):89-102, 1998
                                              17. Beggs I: Pictorial review: imaging of peripheral nerve tumours. Clin Radiol. 52(1):8-17, 1997
                                              18. Kransdorf MJ et al: Imaging of soft tissue tumors, 1st ed. Philadelphia: W.B. Saunders. 240-54, 1997
                                              19. Celli P et al: Primary spinal malignant schwannomas: clinical and prognostic remarks. Acta Neurochir (Wien). 135(1-2):52-5, 1995
                                              20. Bass JC et al: Retroperitoneal plexiform neurofibromas: CT findings. AJR Am J Roentgenol. 163(3):617-20, 1994
                                              21. Suh JS et al: Peripheral (extracranial) nerve tumors: correlation of MR imaging and histologic findings. Radiology. 183(2):341-6, 1992
                                              22. Stull MA et al: Magnetic resonance appearance of peripheral nerve sheath tumors. Skeletal Radiol. 20(1):9-14, 1991
                                              23. Riccardi VM et al: Neurofibrosarcoma as a complication of von Recklinghausen neurofibromatosis. Neurofibromatosis. 2(3):152-65, 1989
                                              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

                                                        TERMINOLOGY

                                                        • Abbreviations

                                                          • Malignant peripheral nerve sheath tumor (MPNST)

                                                        • Synonyms

                                                          • Neurofibrosarcoma
                                                          • Malignant schwannoma
                                                          • Malignant neurilemmoma
                                                          • Neurogenic sarcoma

                                                        • Definitions

                                                          • Soft tissue sarcoma that arises from or differentiates toward cells of peripheral nerve sheath

                                                        IMAGING

                                                        • General Features

                                                          • Radiographic Findings

                                                            • CT Findings

                                                              • MR Findings

                                                                • Angiographic Findings

                                                                  • Nuclear Medicine Findings

                                                                    • Imaging Recommendations

                                                                      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

                                                                                          Selected References

                                                                                          1. Widemann BC et al: Biology and management of undifferentiated pleomorphic sarcoma, myxofibrosarcoma, and malignant peripheral nerve sheath tumors: state of the art and perspectives. J Clin Oncol. 36(2):160-7, 2018
                                                                                          2. Kolberg M et al: Survival meta-analyses for >1800 malignant peripheral nerve sheath tumor patients with and without neurofibromatosis type 1. Neuro Oncol. 15(2):135-47, 2013
                                                                                          3. 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
                                                                                          4. 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
                                                                                          5. Ferner RE et al: [18F.] 2-fluoro-2-deoxy-D-glucose positron emission tomography (FDG PET) as a diagnostic tool for neurofibromatosis 1 (NF1) associated malignant peripheral nerve sheath tumours (MPNSTs): a long-term clinical study. Ann Oncol. 19(2):390-4, 2008
                                                                                          6. Gupta G et al: Malignant peripheral nerve sheath tumors. Neurosurg Clin N Am. 19(4):533-43, v, 2008
                                                                                          7. Amin A et al: Radiotherapy-induced malignant peripheral nerve sheath tumor of the cauda equina. Spine (Phila Pa 1976). 29(21):E506-9, 2004
                                                                                          8. Baehring JM et al: Malignant peripheral nerve sheath tumor: the clinical spectrum and outcome of treatment. Neurology. 61(5):696-8, 2003
                                                                                          9. Kluwe L et al: Constitutional NF1 mutations in neurofibromatosis 1 patients with malignant peripheral nerve sheath tumors. Hum Mutat. 22(5):420, 2003
                                                                                          10. Su W et al: Malignant peripheral nerve sheath tumor cell invasion is facilitated by Src and aberrant CD44 expression. Glia. 42(4):350-8, 2003
                                                                                          11. Asavamongkolkul A et al: Malignant peripheral nerve sheath tumor with neurofibromatosis type 1: a 2-case report and review of the literature. J Med Assoc Thai. 84(2):285-93, 2001
                                                                                          12. Stark AM et al: Malignant peripheral nerve sheath tumours--report of 8 cases and review of the literature. Acta Neurochir (Wien). 143(4):357-63; discussion 363-4, 2001
                                                                                          13. Weiss SW et al: Enzinger and Weiss's soft tissue tumors. 4th ed. St. Louis: CV Mosby. 1209-63, 2001
                                                                                          14. Ferner RE et al: Evaluation of (18)fluorodeoxyglucose positron emission tomography ((18)FDG PET) in the detection of malignant peripheral nerve sheath tumours arising from within plexiform neurofibromas in neurofibromatosis 1. J Neurol Neurosurg Psychiatry. 68(3):353-7, 2000
                                                                                          15. Woodruff JM: Pathology of tumors of the peripheral nerve sheath in type 1 neurofibromatosis. Am J Med Genet. 89(1):23-30, 1999
                                                                                          16. Sangüeza OP et al: Neoplasms with neural differentiation: a review. Part II: Malignant neoplasms. Am J Dermatopathol. 20(1):89-102, 1998
                                                                                          17. Beggs I: Pictorial review: imaging of peripheral nerve tumours. Clin Radiol. 52(1):8-17, 1997
                                                                                          18. Kransdorf MJ et al: Imaging of soft tissue tumors, 1st ed. Philadelphia: W.B. Saunders. 240-54, 1997
                                                                                          19. Celli P et al: Primary spinal malignant schwannomas: clinical and prognostic remarks. Acta Neurochir (Wien). 135(1-2):52-5, 1995
                                                                                          20. Bass JC et al: Retroperitoneal plexiform neurofibromas: CT findings. AJR Am J Roentgenol. 163(3):617-20, 1994
                                                                                          21. Suh JS et al: Peripheral (extracranial) nerve tumors: correlation of MR imaging and histologic findings. Radiology. 183(2):341-6, 1992
                                                                                          22. Stull MA et al: Magnetic resonance appearance of peripheral nerve sheath tumors. Skeletal Radiol. 20(1):9-14, 1991
                                                                                          23. Riccardi VM et al: Neurofibrosarcoma as a complication of von Recklinghausen neurofibromatosis. Neurofibromatosis. 2(3):152-65, 1989