link
Bookmarks
Neurofibromatosis Type 2
Kevin R. Moore, MD
To access 4,300 diagnoses written by the world's leading experts in radiology, please log in or subscribe.Log inSubscribe
0
10
1
0

KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Neurofibromatosis type 2 (NF2), nonsyndromic (NS)
            • Synonyms

              • Bilateral acoustic neurofibromatosis, central neurofibromatosis (both obsolete)
            • Definitions

              • Autosomal dominant disease from chromosomal 22 defect in which all patients develop CNS tumors
              • Mnemonic for NF2 tumors: Multiple inherited schwannomas, meningiomas, and ependymomas (MISME)

            IMAGING

            • General Features

              • Radiographic Findings

                • 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. Dunn IF et al: Congress of Neurological Surgeons systematic review and evidence-based guidelines on the role of imaging in the diagnosis and management of patients with vestibular schwannomas. Neurosurgery. 82(2):E32-4, 2018
                                              2. Lawson McLean AC et al: Growth dynamics of intramedullary spinal tumors in patients with neurofibromatosis type 2. Clin Neurol Neurosurg. 146:130-7, 2016
                                              3. Li P et al: Clinical features of spinal schwannomas in 65 patients with schwannomatosis compared with 831 with solitary schwannomas and 102 with neurofibromatosis Type 2: a retrospective study at a single institution. J Neurosurg Spine. 24(1):145-54, 2016
                                              4. Maiti TK et al: Spinal meningiomas: clinicoradiological factors predicting recurrence and functional outcome. Neurosurg Focus. 41(2):E6, 2016
                                              5. Thomas AK et al: Pediatric schwannomatosis, a rare but distinct form of neurofibromatosis. Pediatr Radiol. 46(3):430-5, 2016
                                              6. Lin AL et al: Advances in the treatment of neurofibromatosis-associated tumours. Nat Rev Clin Oncol. 10(11):616-24, 2013
                                              7. Asthagiri AR et al: Neurofibromatosis type 2. Lancet. 373(9679):1974-86, 2009
                                              8. Evans DG: Neurofibromatosis type 2 (NF2): a clinical and molecular review. Orphanet J Rare Dis. 4:16, 2009
                                              9. Holland K et al: Spinal tumors in neurofibromatosis-2: management considerations - a review. J Clin Neurosci. 16(2):169-77, 2009
                                              10. Patronas NJ et al: Intramedullary and spinal canal tumors in patients with neurofibromatosis 2: MR imaging findings and correlation with genotype. Radiology. 218(2):434-42, 2001
                                              11. Evans DG et al: Neurofibromatosis type 2. J Med Genet. 37(12):897-904, 2000
                                              12. den Bakker MA et al: Expression of the neurofibromatosis type 2 gene in human tissues. J Histochem Cytochem. 47(11):1471-80, 1999
                                              13. Kluwe L et al: Identification of NF2 germ-line mutations and comparison with neurofibromatosis 2 phenotypes. Hum Genet. 98(5):534-8, 1996
                                              Related Anatomy
                                              Loading...
                                              Related Differential Diagnoses
                                              Loading...
                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Top Differential Diagnoses

                                                    • Pathology

                                                      • Diagnostic Checklist

                                                        TERMINOLOGY

                                                        • Abbreviations

                                                          • Neurofibromatosis type 2 (NF2), nonsyndromic (NS)
                                                        • Synonyms

                                                          • Bilateral acoustic neurofibromatosis, central neurofibromatosis (both obsolete)
                                                        • Definitions

                                                          • Autosomal dominant disease from chromosomal 22 defect in which all patients develop CNS tumors
                                                          • Mnemonic for NF2 tumors: Multiple inherited schwannomas, meningiomas, and ependymomas (MISME)

                                                        IMAGING

                                                        • General Features

                                                          • Radiographic Findings

                                                            • 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. Dunn IF et al: Congress of Neurological Surgeons systematic review and evidence-based guidelines on the role of imaging in the diagnosis and management of patients with vestibular schwannomas. Neurosurgery. 82(2):E32-4, 2018
                                                                                          2. Lawson McLean AC et al: Growth dynamics of intramedullary spinal tumors in patients with neurofibromatosis type 2. Clin Neurol Neurosurg. 146:130-7, 2016
                                                                                          3. Li P et al: Clinical features of spinal schwannomas in 65 patients with schwannomatosis compared with 831 with solitary schwannomas and 102 with neurofibromatosis Type 2: a retrospective study at a single institution. J Neurosurg Spine. 24(1):145-54, 2016
                                                                                          4. Maiti TK et al: Spinal meningiomas: clinicoradiological factors predicting recurrence and functional outcome. Neurosurg Focus. 41(2):E6, 2016
                                                                                          5. Thomas AK et al: Pediatric schwannomatosis, a rare but distinct form of neurofibromatosis. Pediatr Radiol. 46(3):430-5, 2016
                                                                                          6. Lin AL et al: Advances in the treatment of neurofibromatosis-associated tumours. Nat Rev Clin Oncol. 10(11):616-24, 2013
                                                                                          7. Asthagiri AR et al: Neurofibromatosis type 2. Lancet. 373(9679):1974-86, 2009
                                                                                          8. Evans DG: Neurofibromatosis type 2 (NF2): a clinical and molecular review. Orphanet J Rare Dis. 4:16, 2009
                                                                                          9. Holland K et al: Spinal tumors in neurofibromatosis-2: management considerations - a review. J Clin Neurosci. 16(2):169-77, 2009
                                                                                          10. Patronas NJ et al: Intramedullary and spinal canal tumors in patients with neurofibromatosis 2: MR imaging findings and correlation with genotype. Radiology. 218(2):434-42, 2001
                                                                                          11. Evans DG et al: Neurofibromatosis type 2. J Med Genet. 37(12):897-904, 2000
                                                                                          12. den Bakker MA et al: Expression of the neurofibromatosis type 2 gene in human tissues. J Histochem Cytochem. 47(11):1471-80, 1999
                                                                                          13. Kluwe L et al: Identification of NF2 germ-line mutations and comparison with neurofibromatosis 2 phenotypes. Hum Genet. 98(5):534-8, 1996