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Neurofibromatosis Type 2
Bernadette L. Koch, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Neurofibromatosis type 2 (NF2)
              • Synonyms

                • Central neurofibromatosis, bilateral acoustic neurofibromatosis, schwannomatosis
                  • Schwannomatosis now considered distinct form of neurofibromatosis, separate from NF1 & NF2
                    • Largely diagnosed in adulthood as peripheral schwannomas with pain control challenges
                    • Genetics not well established
              • Definitions

                • NF2 = inherited syndrome with multiple schwannomas, meningiomas, & ependymomas
                  • Multiple intracranial schwannomas, meningiomas, & ependymomas = "Misme" mneumonic

              IMAGING

              • General Features

                • CT Findings

                  • MR 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

                                        • Image Interpretation Pearls

                                          Selected References

                                          1. Slattery WH: Neurofibromatosis type 2. Otolaryngol Clin North Am. 48(3):443-60, 2015
                                          2. Maniakas A et al: Neurofibromatosis type 2 vestibular schwannoma treatment: a review of the literature, trends, and outcomes. Otol Neurotol. 35(5):889-94, 2014
                                          3. Koontz NA et al: Schwannomatosis: the overlooked neurofibromatosis? AJR Am J Roentgenol. 200(6):W646-53, 2013
                                          4. Sughrue ME et al: A prospective study of hearing preservation in untreated vestibular schwannomas. J Neurosurg. 114(2):381-5, 2011
                                          5. Hsu PW et al: Outcomes of 75 patients over 12 years treated for acoustic neuromas with linear accelerator-based radiosurgery. J Clin Neurosci. 17(5):556-60, 2010
                                          6. Kissil JL et al: What's new in neurofibromatosis? Proceedings from the 2009 NF Conference: new frontiers. Am J Med Genet A. 152A(2):269-83, 2010
                                          7. Mautner VF et al: Bevacizumab induces regression of vestibular schwannomas in patients with neurofibromatosis type 2. Neuro Oncol. 12(1):14-8, 2010
                                          8. Suryanarayanan R et al: Vestibular schwannoma: role of conservative management. J Laryngol Otol. 124(3):251-7, 2010
                                          9. Fisher LM et al: Concordance of bilateral vestibular schwannoma growth and hearing changes in neurofibromatosis 2: neurofibromatosis 2 natural history consortium. Otol Neurotol. 30(6):835-41, 2009
                                          10. Plotkin SR et al: Hearing improvement after bevacizumab in patients with neurofibromatosis type 2. N Engl J Med. 361(4):358-67, 2009
                                          11. Moffat DA et al: Management strategies in neurofibromatosis type 2. Eur Arch Otorhinolaryngol. 260(1):12-8, 2003
                                          12. Brackmann DE et al: Early proactive management of vestibular schwannomas in neurofibromatosis type 2. Neurosurgery. 49(2):274-80; discussion 280-3, 2001
                                          13. Gillespie JE: Imaging in neurofibromatosis type 2: screening using magnetic resonance imaging. Ear Nose Throat J. 78(2):102-3, 106, 108-9, 1999
                                          14. Kiroğlu MM et al: Bilateral acoustic neurofibromatosis with bilateral multicentric facial schwannomas. Eur Arch Otorhinolaryngol. 253(4-5):305-8, 1996
                                          15. Akeson P et al: Radiological investigation of neurofibromatosis type 2. Neuroradiology. 36(2):107-10, 1994
                                          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

                                                        • Central neurofibromatosis, bilateral acoustic neurofibromatosis, schwannomatosis
                                                          • Schwannomatosis now considered distinct form of neurofibromatosis, separate from NF1 & NF2
                                                            • Largely diagnosed in adulthood as peripheral schwannomas with pain control challenges
                                                            • Genetics not well established
                                                      • Definitions

                                                        • NF2 = inherited syndrome with multiple schwannomas, meningiomas, & ependymomas
                                                          • Multiple intracranial schwannomas, meningiomas, & ependymomas = "Misme" mneumonic

                                                      IMAGING

                                                      • General Features

                                                        • CT Findings

                                                          • MR 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

                                                                                • Image Interpretation Pearls

                                                                                  Selected References

                                                                                  1. Slattery WH: Neurofibromatosis type 2. Otolaryngol Clin North Am. 48(3):443-60, 2015
                                                                                  2. Maniakas A et al: Neurofibromatosis type 2 vestibular schwannoma treatment: a review of the literature, trends, and outcomes. Otol Neurotol. 35(5):889-94, 2014
                                                                                  3. Koontz NA et al: Schwannomatosis: the overlooked neurofibromatosis? AJR Am J Roentgenol. 200(6):W646-53, 2013
                                                                                  4. Sughrue ME et al: A prospective study of hearing preservation in untreated vestibular schwannomas. J Neurosurg. 114(2):381-5, 2011
                                                                                  5. Hsu PW et al: Outcomes of 75 patients over 12 years treated for acoustic neuromas with linear accelerator-based radiosurgery. J Clin Neurosci. 17(5):556-60, 2010
                                                                                  6. Kissil JL et al: What's new in neurofibromatosis? Proceedings from the 2009 NF Conference: new frontiers. Am J Med Genet A. 152A(2):269-83, 2010
                                                                                  7. Mautner VF et al: Bevacizumab induces regression of vestibular schwannomas in patients with neurofibromatosis type 2. Neuro Oncol. 12(1):14-8, 2010
                                                                                  8. Suryanarayanan R et al: Vestibular schwannoma: role of conservative management. J Laryngol Otol. 124(3):251-7, 2010
                                                                                  9. Fisher LM et al: Concordance of bilateral vestibular schwannoma growth and hearing changes in neurofibromatosis 2: neurofibromatosis 2 natural history consortium. Otol Neurotol. 30(6):835-41, 2009
                                                                                  10. Plotkin SR et al: Hearing improvement after bevacizumab in patients with neurofibromatosis type 2. N Engl J Med. 361(4):358-67, 2009
                                                                                  11. Moffat DA et al: Management strategies in neurofibromatosis type 2. Eur Arch Otorhinolaryngol. 260(1):12-8, 2003
                                                                                  12. Brackmann DE et al: Early proactive management of vestibular schwannomas in neurofibromatosis type 2. Neurosurgery. 49(2):274-80; discussion 280-3, 2001
                                                                                  13. Gillespie JE: Imaging in neurofibromatosis type 2: screening using magnetic resonance imaging. Ear Nose Throat J. 78(2):102-3, 106, 108-9, 1999
                                                                                  14. Kiroğlu MM et al: Bilateral acoustic neurofibromatosis with bilateral multicentric facial schwannomas. Eur Arch Otorhinolaryngol. 253(4-5):305-8, 1996
                                                                                  15. Akeson P et al: Radiological investigation of neurofibromatosis type 2. Neuroradiology. 36(2):107-10, 1994