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Spine
Diagnosis
Neoplasms, Cysts, and Other Masses
Neoplasms
Intradural Extramedullary
Neurofibroma
Neurofibroma
Jeffrey S. Ross, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Neurofibroma (NF)

              • Synonyms

                • Nerve sheath tumor (NST)
                  • Category of NST also includes schwannomas

              • Definitions

                • Benign neoplasm containing Schwann cells, fibroblasts, myxoid material, and peripheral nerve fibers without true capsule (WHO grade 1)

              IMAGING

              • General Features

                • Radiographic Findings

                  • CT Findings

                    • MR Findings

                      • Nuclear Medicine Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Staging, Grading, & Classification

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Ledbetter LN et al: Imaging of intraspinal tumors. Radiol Clin North Am. 57(2):341-57, 2019
                                              2. Stewart DR et al: Care of adults with neurofibromatosis type 1: a clinical practice resource of the American College of Medical Genetics and Genomics (ACMG). Genet Med. 20(7):671-82, 2018
                                              3. Merhemic Z et al: Neuroimaging of spinal tumors. Magn Reson Imaging Clin N Am. 24(3):563-79, 2016
                                              4. Simou N et al: Plexiform neurofibroma of the cauda equina: a case report and review of the literature. Int J Surg Pathol. 16(1):78-80, 2008
                                              5. Sehgal VN et al: Solitary plexiform neurofibroma(s): role of magnetic resonance imaging. Skinmed. 6(2):99-100, 2007
                                              6. Brenner W et al: Prognostic relevance of FDG PET in patients with neurofibromatosis type-1 and malignant peripheral nerve sheath tumours. Eur J Nucl Med Mol Imaging. 33(4):428-32, 2006
                                              7. Cardona S et al: Evaluation of F18-deoxyglucose positron emission tomography (FDG-PET) to assess the nature of neurogenic tumours. Eur J Surg Oncol. 29(6):536-41, 2003
                                              8. Garg S et al: Quadriplegia in a 10 year-old boy due to multiple cervical neurofibromas. Spine (Phila Pa 1976). 28(17):E339-43, 2003
                                              9. Khong PL et al: MR imaging of spinal tumors in children with neurofibromatosis 1. AJR Am J Roentgenol. 180(2):413-7, 2003
                                              10. Mautner VF et al: Malignant peripheral nerve sheath tumours in neurofibromatosis type 1: MRI supports the diagnosis of malignant plexiform neurofibroma. Neuroradiology. 45(9):618-25, 2003
                                              11. Simoens WA et al: MR features of peripheral nerve sheath tumors: can a calculated index compete with radiologist's experience? Eur Radiol. 11(2):250-7, 2001
                                              12. 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
                                              13. Woodruff JM et al: Neurofibroma. In Kleihues P, Cavenee WK. Tumors of the Nervous System. Lyon: IARC Press. 167-8, 2000
                                              14. Murphey MD et al: From the archives of the AFIP. Imaging of musculoskeletal neurogenic tumors: radiologic-pathologic correlation. Radiographics. 19(5):1253-80, 1999
                                              15. Thakkar SD et al: Spinal tumours in neurofibromatosis type 1: an MRI study of frequency, multiplicity and variety. Neuroradiology. 41(9):625-9, 1999
                                              16. Klekamp J et al: Surgery of spinal nerve sheath tumors with special reference to neurofibromatosis. Neurosurgery. 42(2):279-89; discussion 289-90, 1998
                                              17. Shariff SY et al: Intraspinal tumours in children--clinical presentation. Ir Med J. 90(7):264-5, 1997
                                              18. Yagi T et al: Intramedullary spinal cord tumour associated with neurofibromatosis type 1. Acta Neurochir (Wien). 139(11):1055-60, 1997
                                              19. Tatagiba M et al: Involvement of spinal nerves in neurofibromatosis. Neurosurg Rev. 17(1):43-9, 1994
                                              20. Clinchot DM et al: The spectrum of neurofibromatosis: neurologic manifestations with malignant transformation. J Am Paraplegia Soc. 16(2):81-3, 1993
                                              21. Shu HH et al: Neurofibromatosis: MR imaging findings involving the head and spine. AJR Am J Roentgenol. 160(1):159-64, 1993
                                              22. Egelhoff JC et al: Spinal MR findings in neurofibromatosis types 1 and 2. AJNR Am J Neuroradiol. 13(4):1071-7, 1992
                                              23. Li MH et al: MR imaging of spinal neurofibromatosis. Acta Radiol. 32(4):279-85, 1991
                                              24. Ros PR et al: Plexiform neurofibroma of the pelvis: CT and MRI findings. Magn Reson Imaging. 9(3):463-5, 1991
                                              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

                                                          • Abbreviations

                                                            • Neurofibroma (NF)

                                                          • Synonyms

                                                            • Nerve sheath tumor (NST)
                                                              • Category of NST also includes schwannomas

                                                          • Definitions

                                                            • Benign neoplasm containing Schwann cells, fibroblasts, myxoid material, and peripheral nerve fibers without true capsule (WHO grade 1)

                                                          IMAGING

                                                          • General Features

                                                            • Radiographic Findings

                                                              • CT Findings

                                                                • MR Findings

                                                                  • Nuclear Medicine Findings

                                                                    • Imaging Recommendations

                                                                      DIFFERENTIAL DIAGNOSIS

                                                                        PATHOLOGY

                                                                        • General Features

                                                                          • Staging, Grading, & Classification

                                                                            • Microscopic Features

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Ledbetter LN et al: Imaging of intraspinal tumors. Radiol Clin North Am. 57(2):341-57, 2019
                                                                                          2. Stewart DR et al: Care of adults with neurofibromatosis type 1: a clinical practice resource of the American College of Medical Genetics and Genomics (ACMG). Genet Med. 20(7):671-82, 2018
                                                                                          3. Merhemic Z et al: Neuroimaging of spinal tumors. Magn Reson Imaging Clin N Am. 24(3):563-79, 2016
                                                                                          4. Simou N et al: Plexiform neurofibroma of the cauda equina: a case report and review of the literature. Int J Surg Pathol. 16(1):78-80, 2008
                                                                                          5. Sehgal VN et al: Solitary plexiform neurofibroma(s): role of magnetic resonance imaging. Skinmed. 6(2):99-100, 2007
                                                                                          6. Brenner W et al: Prognostic relevance of FDG PET in patients with neurofibromatosis type-1 and malignant peripheral nerve sheath tumours. Eur J Nucl Med Mol Imaging. 33(4):428-32, 2006
                                                                                          7. Cardona S et al: Evaluation of F18-deoxyglucose positron emission tomography (FDG-PET) to assess the nature of neurogenic tumours. Eur J Surg Oncol. 29(6):536-41, 2003
                                                                                          8. Garg S et al: Quadriplegia in a 10 year-old boy due to multiple cervical neurofibromas. Spine (Phila Pa 1976). 28(17):E339-43, 2003
                                                                                          9. Khong PL et al: MR imaging of spinal tumors in children with neurofibromatosis 1. AJR Am J Roentgenol. 180(2):413-7, 2003
                                                                                          10. Mautner VF et al: Malignant peripheral nerve sheath tumours in neurofibromatosis type 1: MRI supports the diagnosis of malignant plexiform neurofibroma. Neuroradiology. 45(9):618-25, 2003
                                                                                          11. Simoens WA et al: MR features of peripheral nerve sheath tumors: can a calculated index compete with radiologist's experience? Eur Radiol. 11(2):250-7, 2001
                                                                                          12. 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
                                                                                          13. Woodruff JM et al: Neurofibroma. In Kleihues P, Cavenee WK. Tumors of the Nervous System. Lyon: IARC Press. 167-8, 2000
                                                                                          14. Murphey MD et al: From the archives of the AFIP. Imaging of musculoskeletal neurogenic tumors: radiologic-pathologic correlation. Radiographics. 19(5):1253-80, 1999
                                                                                          15. Thakkar SD et al: Spinal tumours in neurofibromatosis type 1: an MRI study of frequency, multiplicity and variety. Neuroradiology. 41(9):625-9, 1999
                                                                                          16. Klekamp J et al: Surgery of spinal nerve sheath tumors with special reference to neurofibromatosis. Neurosurgery. 42(2):279-89; discussion 289-90, 1998
                                                                                          17. Shariff SY et al: Intraspinal tumours in children--clinical presentation. Ir Med J. 90(7):264-5, 1997
                                                                                          18. Yagi T et al: Intramedullary spinal cord tumour associated with neurofibromatosis type 1. Acta Neurochir (Wien). 139(11):1055-60, 1997
                                                                                          19. Tatagiba M et al: Involvement of spinal nerves in neurofibromatosis. Neurosurg Rev. 17(1):43-9, 1994
                                                                                          20. Clinchot DM et al: The spectrum of neurofibromatosis: neurologic manifestations with malignant transformation. J Am Paraplegia Soc. 16(2):81-3, 1993
                                                                                          21. Shu HH et al: Neurofibromatosis: MR imaging findings involving the head and spine. AJR Am J Roentgenol. 160(1):159-64, 1993
                                                                                          22. Egelhoff JC et al: Spinal MR findings in neurofibromatosis types 1 and 2. AJNR Am J Neuroradiol. 13(4):1071-7, 1992
                                                                                          23. Li MH et al: MR imaging of spinal neurofibromatosis. Acta Radiol. 32(4):279-85, 1991
                                                                                          24. Ros PR et al: Plexiform neurofibroma of the pelvis: CT and MRI findings. Magn Reson Imaging. 9(3):463-5, 1991
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