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Neurofibromatosis Type 2, Spine
Kevin R. Moore, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Neurofibromatosis type 2 (NF2)
            • Synonyms

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

              • Rare autosomal dominant disease from chromosome 22 defect in which all patients develop CNS tumors

            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

                                            Selected References

                                            1. Wu L et al: Spinal intradural malignant peripheral nerve sheath tumor in a child with neurofibromatosis type 2: the first reported case and literature review. Turk Neurosurg. 24(1):135-9, 2014
                                            2. Bydon M et al: Multiple primary intramedullary ependymomas: a case report and review of the literature. Spine J. 13(10):1379-86, 2013
                                            3. Aguilera DG et al: Neurofibromatosis-2 and spinal cord ependymomas: Report of two cases and review of the literature. Childs Nerv Syst. 27(5):757-64, 2011
                                            4. Gonzalvo A et al: Schwannomatosis, sporadic schwannomatosis, and familial schwannomatosis: a surgical series with long-term follow-up. Clinical article. J Neurosurg. 114(3):756-62, 2011
                                            5. Plotkin SR et al: Spinal ependymomas in neurofibromatosis Type 2: a retrospective analysis of 55 patients. J Neurosurg Spine. 14(4):543-7, 2011
                                            6. Asthagiri AR et al: Neurofibromatosis type 2. Lancet. 373(9679):1974-86, 2009
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Top Differential Diagnoses

                                                  • Pathology

                                                    • Diagnostic Checklist

                                                      TERMINOLOGY

                                                      • Abbreviations

                                                        • Neurofibromatosis type 2 (NF2)
                                                      • Synonyms

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

                                                        • Rare autosomal dominant disease from chromosome 22 defect in which all patients develop CNS tumors

                                                      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

                                                                                      Selected References

                                                                                      1. Wu L et al: Spinal intradural malignant peripheral nerve sheath tumor in a child with neurofibromatosis type 2: the first reported case and literature review. Turk Neurosurg. 24(1):135-9, 2014
                                                                                      2. Bydon M et al: Multiple primary intramedullary ependymomas: a case report and review of the literature. Spine J. 13(10):1379-86, 2013
                                                                                      3. Aguilera DG et al: Neurofibromatosis-2 and spinal cord ependymomas: Report of two cases and review of the literature. Childs Nerv Syst. 27(5):757-64, 2011
                                                                                      4. Gonzalvo A et al: Schwannomatosis, sporadic schwannomatosis, and familial schwannomatosis: a surgical series with long-term follow-up. Clinical article. J Neurosurg. 114(3):756-62, 2011
                                                                                      5. Plotkin SR et al: Spinal ependymomas in neurofibromatosis Type 2: a retrospective analysis of 55 patients. J Neurosurg Spine. 14(4):543-7, 2011
                                                                                      6. Asthagiri AR et al: Neurofibromatosis type 2. Lancet. 373(9679):1974-86, 2009