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Neurofibromatosis Type 2, Brain
Luke L. Linscott, MDSurjith Vattoth, MD, FRCR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Neurofibromatosis type 2 (NF2)
              • Synonyms

                • Acoustic neurofibromatosis, central neurofibromatosis
                • Multiple intracranial schwannomas, meningiomas, and ependymomas (MISME)
              • Definitions

                • Familial cancer syndrome causing multiple cranial nerve (CN) schwannomas, meningiomas, and spinal ependymomas

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Nonvascular Interventions

                      • 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

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Yao L et al: Therapy of sporadic and NF2-related vestibular schwannoma. Cancers (Basel). 12(4):835, 2020
                                              2. Nguyen T et al: Surgery versus stereotactic radiosurgery for the treatment of multiple meningiomas in neurofibromatosis type 2: illustrative case and systematic review. Neurosurg Rev. 42(1):85-96, 2019
                                              3. Sverak P et al: Bevacizumab for hearing preservation in neurofibromatosis type 2: Emphasis on patient-reported outcomes and toxicities. Otolaryngol Head Neck Surg. 160(3):526-32, 2019
                                              4. Kaul V et al: Management of vestibular schwannoma (including NF2): facial nerve considerations. Otolaryngol Clin North Am. 51(6):1193-212, 2018
                                              5. Morris KA et al: Comparing the sensitivity of linear and volumetric MRI measurements to detect changes in the size of vestibular schwannomas in patients with neurofibromatosis type 2 on bevacizumab treatment. Br J Radiol. 89(1065):20160110, 2016
                                              6. Evans DG et al: Bilateral vestibular schwannomas in older patients: NF2 or chance? J Med Genet. 52(6):422-4, 2015
                                              7. Jethanamest D et al: Conservative management of vestibular schwannoma: Predictors of growth and hearing. Laryngoscope. 125(9):2163-8, 2015
                                              8. Ferner RE et al: Longitudinal evaluation of quality of life in 288 patients with neurofibromatosis 2. J Neurol. 261(5):963-9, 2014
                                              9. Giovannini M et al: mTORC1 inhibition delays growth of neurofibromatosis type 2 schwannoma. Neuro Oncol. 16(4):493-504, 2014
                                              10. Matsuo M et al: Characterization of early onset neurofibromatosis type 2. Brain Dev. 36(2):148-52, 2014
                                              11. Plotkin SR et al: Natural history of vestibular schwannoma growth and hearing decline in newly diagnosed neurofibromatosis type 2 patients. Otol Neurotol. 35(1):e50-6, 2014
                                              12. Vargas WS et al: Incidental parenchymal magnetic resonance imaging findings in the brains of patients with neurofibromatosis type 2. Neuroimage Clin. 4:258-65, 2014
                                              13. Goutagny S et al: Long-term follow-up of 287 meningiomas in neurofibromatosis type 2 patients: clinical, radiological, and molecular features. Neuro Oncol. 14(8):1090-6, 2012
                                              14. Hanemann CO: Magic but treatable? Tumours due to loss of merlin. Brain. 131(Pt 3):606-15, 2008
                                              15. Fisher LM et al: Distribution of nonvestibular cranial nerve schwannomas in neurofibromatosis 2. Otol Neurotol. 28(8):1083-90, 2007
                                              16. Bosch MM et al: Optic nerve sheath meningiomas in patients with neurofibromatosis type 2. Arch Ophthalmol. 124(3):379-85, 2006
                                              17. Omeis I et al: Meningioangiomatosis associated with neurofibromatosis: report of 2 cases in a single family and review of the literature. Surg Neurol. 65(6):595-603, 2006
                                              18. Neff BA et al: Current concepts in the evaluation and treatment of neurofibromatosis type II. Otolaryngol Clin North Am. 38(4):671-84, ix, 2005
                                              19. Ruggieri M et al: Earliest clinical manifestations and natural history of neurofibromatosis type 2 (NF2) in childhood: a study of 24 patients. Neuropediatrics. 36(1):21-34, 2005
                                              20. Otsuka G et al: Age at symptom onset and long-term survival in patients with neurofibromatosis Type 2. J Neurosurg. 99(3):480-3, 2003
                                              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

                                                            • Neurofibromatosis type 2 (NF2)
                                                          • Synonyms

                                                            • Acoustic neurofibromatosis, central neurofibromatosis
                                                            • Multiple intracranial schwannomas, meningiomas, and ependymomas (MISME)
                                                          • Definitions

                                                            • Familial cancer syndrome causing multiple cranial nerve (CN) schwannomas, meningiomas, and spinal ependymomas

                                                          IMAGING

                                                          • General Features

                                                            • CT Findings

                                                              • MR Findings

                                                                • Nonvascular Interventions

                                                                  • 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

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Yao L et al: Therapy of sporadic and NF2-related vestibular schwannoma. Cancers (Basel). 12(4):835, 2020
                                                                                          2. Nguyen T et al: Surgery versus stereotactic radiosurgery for the treatment of multiple meningiomas in neurofibromatosis type 2: illustrative case and systematic review. Neurosurg Rev. 42(1):85-96, 2019
                                                                                          3. Sverak P et al: Bevacizumab for hearing preservation in neurofibromatosis type 2: Emphasis on patient-reported outcomes and toxicities. Otolaryngol Head Neck Surg. 160(3):526-32, 2019
                                                                                          4. Kaul V et al: Management of vestibular schwannoma (including NF2): facial nerve considerations. Otolaryngol Clin North Am. 51(6):1193-212, 2018
                                                                                          5. Morris KA et al: Comparing the sensitivity of linear and volumetric MRI measurements to detect changes in the size of vestibular schwannomas in patients with neurofibromatosis type 2 on bevacizumab treatment. Br J Radiol. 89(1065):20160110, 2016
                                                                                          6. Evans DG et al: Bilateral vestibular schwannomas in older patients: NF2 or chance? J Med Genet. 52(6):422-4, 2015
                                                                                          7. Jethanamest D et al: Conservative management of vestibular schwannoma: Predictors of growth and hearing. Laryngoscope. 125(9):2163-8, 2015
                                                                                          8. Ferner RE et al: Longitudinal evaluation of quality of life in 288 patients with neurofibromatosis 2. J Neurol. 261(5):963-9, 2014
                                                                                          9. Giovannini M et al: mTORC1 inhibition delays growth of neurofibromatosis type 2 schwannoma. Neuro Oncol. 16(4):493-504, 2014
                                                                                          10. Matsuo M et al: Characterization of early onset neurofibromatosis type 2. Brain Dev. 36(2):148-52, 2014
                                                                                          11. Plotkin SR et al: Natural history of vestibular schwannoma growth and hearing decline in newly diagnosed neurofibromatosis type 2 patients. Otol Neurotol. 35(1):e50-6, 2014
                                                                                          12. Vargas WS et al: Incidental parenchymal magnetic resonance imaging findings in the brains of patients with neurofibromatosis type 2. Neuroimage Clin. 4:258-65, 2014
                                                                                          13. Goutagny S et al: Long-term follow-up of 287 meningiomas in neurofibromatosis type 2 patients: clinical, radiological, and molecular features. Neuro Oncol. 14(8):1090-6, 2012
                                                                                          14. Hanemann CO: Magic but treatable? Tumours due to loss of merlin. Brain. 131(Pt 3):606-15, 2008
                                                                                          15. Fisher LM et al: Distribution of nonvestibular cranial nerve schwannomas in neurofibromatosis 2. Otol Neurotol. 28(8):1083-90, 2007
                                                                                          16. Bosch MM et al: Optic nerve sheath meningiomas in patients with neurofibromatosis type 2. Arch Ophthalmol. 124(3):379-85, 2006
                                                                                          17. Omeis I et al: Meningioangiomatosis associated with neurofibromatosis: report of 2 cases in a single family and review of the literature. Surg Neurol. 65(6):595-603, 2006
                                                                                          18. Neff BA et al: Current concepts in the evaluation and treatment of neurofibromatosis type II. Otolaryngol Clin North Am. 38(4):671-84, ix, 2005
                                                                                          19. Ruggieri M et al: Earliest clinical manifestations and natural history of neurofibromatosis type 2 (NF2) in childhood: a study of 24 patients. Neuropediatrics. 36(1):21-34, 2005
                                                                                          20. Otsuka G et al: Age at symptom onset and long-term survival in patients with neurofibromatosis Type 2. J Neurosurg. 99(3):480-3, 2003