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Nonvestibular Schwannoma
Nicholas A. Koontz, MD; Anne G. Osborn, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          TERMINOLOGY

          • Abbreviations

            • Nonvestibular schwannoma (nVS)
          • Synonyms

            • Neurilemmoma, neurinoma
          • Definitions

            • Benign encapsulated nerve sheath tumor composed of differentiated neoplastic Schwann cells

          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

                                    • Consider

                                      Selected References

                                      1. Sanmillán JL et al: Supratentorial brain schwannomas: an uncommon location for a common tumour. Br J Neurosurg. 28(1):25-8, 2014
                                      2. Bondi S et al: Non-vestibular head and neck schwannomas: a 10-year experience. Eur Arch Otorhinolaryngol. 270(8):2365-9, 2013
                                      3. Luo W et al: Intracranial intraparenchymal and intraventricular schwannomas: report of 18 cases. Clin Neurol Neurosurg. 115(7):1052-7, 2013
                                      4. Rousseau G et al: SMARCB1/INI1 germline mutations contribute to 10% of sporadic schwannomatosis. BMC Neurol. 11:9, 2011
                                      5. Sughrue ME et al: The natural history of untreated sporadic vestibular schwannomas: a comprehensive review of hearing outcomes. J Neurosurg. 112(1):163-7, 2010
                                      6. Ambekar S et al: Frontal intraparenchymal Schwannoma--case report and review of literature. Br J Neurosurg. 23(1):86-9, 2009
                                      7. Asthagiri AR et al: Neurofibromatosis type 2. Lancet. 373(9679):1974-86, 2009
                                      8. Azarpira N et al: Cytologic findings in pigmented melanotic schwannoma: a case report. Acta Cytol. 53(1):113-5, 2009
                                      9. Godefroy WP et al: Surgery for large vestibular schwannoma: residual tumor and outcome. Otol Neurotol. 30(5):629-34, 2009
                                      10. Ito E et al: Factors predicting growth of vestibular schwannoma in neurofibromatosis type 2. Neurosurg Rev. 32(4):425-33, 2009
                                      11. James MF et al: NF2/merlin is a novel negative regulator of mTOR complex 1, and activation of mTORC1 is associated with meningioma and schwannoma growth. Mol Cell Biol. 29(15):4250-61, 2009
                                      12. Kano H et al: Stereotactic radiosurgery for trigeminal schwannoma: tumor control and functional preservation Clinical article. J Neurosurg. 110(3):553-8, 2009
                                      13. Lobato-Polo J et al: Gamma knife radiosurgery in younger patients with vestibular schwannomas. Neurosurgery. 65(2):294-300; discussion 300-1, 2009
                                      14. Mann W et al: Diagnosis and therapy of vestibular schwannoma. Expert Rev Neurother. 9(8):1219-32, 2009
                                      15. Marzo SJ et al: Facial nerve schwannoma. Curr Opin Otolaryngol Head Neck Surg. 17(5):346-50, 2009
                                      16. Mathiesen T et al: Hypoglossal schwannoma-successful reinnervation and functional recovery of the tongue following tumour removal and nerve grafting. Acta Neurochir (Wien). 151(7):837-41; discussion 841, 2009
                                      17. Menkü A et al: Atypical intracerebral schwannoma mimicking glial tumor: case report. Turk Neurosurg. 19(1):82-5, 2009
                                      18. Mukherjee J et al: Human schwannomas express activated platelet-derived growth factor receptors and c-kit and are growth inhibited by Gleevec (Imatinib Mesylate). Cancer Res. 69(12):5099-107, 2009
                                      19. Beauchesne P et al: Malignant nerve sheath tumor of the right cerebral peduncle: case report. Neurosurgery. 54(2):500-3; discussion 503-4, 2004
                                      20. Beaulieu S et al: Positron emission tomography of schwannomas: emphasizing its potential in preoperative planning. AJR Am J Roentgenol. 182(4):971-4, 2004
                                      21. Kurokawa R et al: Spinal accessory schwannoma mimicking a tumor of the fourth ventricle: case report. Neurosurgery. 54(2):510-4; discussion 514, 2004
                                      22. Luo B et al: Neuroradiological findings of intracranial schwannomas not arising from the stems of cranial nerves. Br J Radiol. 77(924):1016-21, 2004
                                      23. Reynolds DL Jr et al: Sonographic characteristics of peripheral nerve sheath tumors. AJR Am J Roentgenol. 182(3):741-4, 2004
                                      24. Chng SM et al: Intracerebral schwannoma--a rare cause of epilepsy. Ann Acad Med Singapore. 32(4):547-9, 2003
                                      25. Erdogan E et al: Schwannoma of the lateral ventricle: eight-year follow-up and literature review. Minim Invasive Neurosurg. 46(1):50-3, 2003
                                      26. Lin J et al: Intraparenchymal schwannoma of the medulla oblongata. Case report. J Neurosurg. 98(3):621-4, 2003
                                      27. Louis E et al: Intra-cerebral schwannoma simulating glioma. J Neurooncol. 64(3):279-82, 2003
                                      28. Sener RN: Diffusion magnetic resonance imaging of solid vestibular schwannomas. J Comput Assist Tomogr. 27(2):249-52, 2003
                                      29. Amador AR et al: Olfactory schwannoma. Eur Radiol. 12(4):742-4, 2002
                                      30. Figarella-Branger D et al: Pituicytomas, a mis-diagnosed benign tumor of the neurohypophysis: report of three cases. Acta Neuropathol (Berl). 104(3):313-9, 2002
                                      31. Huang TW et al: Differentiation between cerebellopontine angle tumors in cancer patients. Otol Neurotol. 23(6):975-9, 2002
                                      32. Nakayama K et al: Supratentorial convexity leptomeningeal schwannoma: case report. Neurosurgery. 51(5):1295-7; discussion 1298, 2002
                                      33. Sarma S et al: Nonvestibular schwannomas of the brain: a 7-year experience. Neurosurgery. 50(3):437-48; discussion 438-9, 2002
                                      34. Sperfeld AD et al: Occurrence and characterization of peripheral nerve involvement in neurofibromatosis type 2. Brain. 125(Pt 5):996-1004, 2002
                                      35. Suh YL et al: Tumors of the central nervous system in Korea: a multicenter study of 3221 cases. J Neurooncol. 56(3):251-9, 2002
                                      36. Ture U et al: Infratentorial lateral supracerebellar approach for trochlear nerve schwannoma. J Clin Neurosci. 9(5):595-8, 2002
                                      37. Whee SM et al: Intrasellar schwannoma mimicking pituitary adenoma: a case report. J Korean Med Sci. 17(1):147-50, 2002
                                      38. Janse AJ et al: Neurofibromatosis type 2 diagnosed in the absence of vestibular schwannomas. A case report and guidelines for a screening protocol for children at risk. Eur J Pediatr. 160(7):439-43, 2001
                                      39. Tan TC et al: Subfrontal schwannoma masquerading as meningioma. Singapore Med J. 42(6):275-7, 2001
                                      40. Zabel A et al: Management of benign cranial nonacoustic schwannomas by fractionated stereotactic radiotherapy. Int J Cancer. 96(6):356-62, 2001
                                      41. Eldevik OP et al: Imaging findings in schwannomas of the jugular foramen. AJNR Am J Neuroradiol. 21(6):1139-44, 2000
                                      42. Lingawi SS: Oculomotor nerve schwannoma: MRI appearance. Clin Imaging. 24(2):86-8, 2000
                                      43. Woodruff JM et al: Schwannoma. In Kleihues P et al: Tumours of the Nervous System. Lyon: IARC Press. 164-6, 2000
                                      Related Anatomy
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                                      Related Differential Diagnoses
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                                      References
                                      Tables

                                      Tables

                                      KEY FACTS

                                      • Terminology

                                        • Imaging

                                          • Top Differential Diagnoses

                                            • Pathology

                                              TERMINOLOGY

                                              • Abbreviations

                                                • Nonvestibular schwannoma (nVS)
                                              • Synonyms

                                                • Neurilemmoma, neurinoma
                                              • Definitions

                                                • Benign encapsulated nerve sheath tumor composed of differentiated neoplastic Schwann cells

                                              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

                                                                        • Consider

                                                                          Selected References

                                                                          1. Sanmillán JL et al: Supratentorial brain schwannomas: an uncommon location for a common tumour. Br J Neurosurg. 28(1):25-8, 2014
                                                                          2. Bondi S et al: Non-vestibular head and neck schwannomas: a 10-year experience. Eur Arch Otorhinolaryngol. 270(8):2365-9, 2013
                                                                          3. Luo W et al: Intracranial intraparenchymal and intraventricular schwannomas: report of 18 cases. Clin Neurol Neurosurg. 115(7):1052-7, 2013
                                                                          4. Rousseau G et al: SMARCB1/INI1 germline mutations contribute to 10% of sporadic schwannomatosis. BMC Neurol. 11:9, 2011
                                                                          5. Sughrue ME et al: The natural history of untreated sporadic vestibular schwannomas: a comprehensive review of hearing outcomes. J Neurosurg. 112(1):163-7, 2010
                                                                          6. Ambekar S et al: Frontal intraparenchymal Schwannoma--case report and review of literature. Br J Neurosurg. 23(1):86-9, 2009
                                                                          7. Asthagiri AR et al: Neurofibromatosis type 2. Lancet. 373(9679):1974-86, 2009
                                                                          8. Azarpira N et al: Cytologic findings in pigmented melanotic schwannoma: a case report. Acta Cytol. 53(1):113-5, 2009
                                                                          9. Godefroy WP et al: Surgery for large vestibular schwannoma: residual tumor and outcome. Otol Neurotol. 30(5):629-34, 2009
                                                                          10. Ito E et al: Factors predicting growth of vestibular schwannoma in neurofibromatosis type 2. Neurosurg Rev. 32(4):425-33, 2009
                                                                          11. James MF et al: NF2/merlin is a novel negative regulator of mTOR complex 1, and activation of mTORC1 is associated with meningioma and schwannoma growth. Mol Cell Biol. 29(15):4250-61, 2009
                                                                          12. Kano H et al: Stereotactic radiosurgery for trigeminal schwannoma: tumor control and functional preservation Clinical article. J Neurosurg. 110(3):553-8, 2009
                                                                          13. Lobato-Polo J et al: Gamma knife radiosurgery in younger patients with vestibular schwannomas. Neurosurgery. 65(2):294-300; discussion 300-1, 2009
                                                                          14. Mann W et al: Diagnosis and therapy of vestibular schwannoma. Expert Rev Neurother. 9(8):1219-32, 2009
                                                                          15. Marzo SJ et al: Facial nerve schwannoma. Curr Opin Otolaryngol Head Neck Surg. 17(5):346-50, 2009
                                                                          16. Mathiesen T et al: Hypoglossal schwannoma-successful reinnervation and functional recovery of the tongue following tumour removal and nerve grafting. Acta Neurochir (Wien). 151(7):837-41; discussion 841, 2009
                                                                          17. Menkü A et al: Atypical intracerebral schwannoma mimicking glial tumor: case report. Turk Neurosurg. 19(1):82-5, 2009
                                                                          18. Mukherjee J et al: Human schwannomas express activated platelet-derived growth factor receptors and c-kit and are growth inhibited by Gleevec (Imatinib Mesylate). Cancer Res. 69(12):5099-107, 2009
                                                                          19. Beauchesne P et al: Malignant nerve sheath tumor of the right cerebral peduncle: case report. Neurosurgery. 54(2):500-3; discussion 503-4, 2004
                                                                          20. Beaulieu S et al: Positron emission tomography of schwannomas: emphasizing its potential in preoperative planning. AJR Am J Roentgenol. 182(4):971-4, 2004
                                                                          21. Kurokawa R et al: Spinal accessory schwannoma mimicking a tumor of the fourth ventricle: case report. Neurosurgery. 54(2):510-4; discussion 514, 2004
                                                                          22. Luo B et al: Neuroradiological findings of intracranial schwannomas not arising from the stems of cranial nerves. Br J Radiol. 77(924):1016-21, 2004
                                                                          23. Reynolds DL Jr et al: Sonographic characteristics of peripheral nerve sheath tumors. AJR Am J Roentgenol. 182(3):741-4, 2004
                                                                          24. Chng SM et al: Intracerebral schwannoma--a rare cause of epilepsy. Ann Acad Med Singapore. 32(4):547-9, 2003
                                                                          25. Erdogan E et al: Schwannoma of the lateral ventricle: eight-year follow-up and literature review. Minim Invasive Neurosurg. 46(1):50-3, 2003
                                                                          26. Lin J et al: Intraparenchymal schwannoma of the medulla oblongata. Case report. J Neurosurg. 98(3):621-4, 2003
                                                                          27. Louis E et al: Intra-cerebral schwannoma simulating glioma. J Neurooncol. 64(3):279-82, 2003
                                                                          28. Sener RN: Diffusion magnetic resonance imaging of solid vestibular schwannomas. J Comput Assist Tomogr. 27(2):249-52, 2003
                                                                          29. Amador AR et al: Olfactory schwannoma. Eur Radiol. 12(4):742-4, 2002
                                                                          30. Figarella-Branger D et al: Pituicytomas, a mis-diagnosed benign tumor of the neurohypophysis: report of three cases. Acta Neuropathol (Berl). 104(3):313-9, 2002
                                                                          31. Huang TW et al: Differentiation between cerebellopontine angle tumors in cancer patients. Otol Neurotol. 23(6):975-9, 2002
                                                                          32. Nakayama K et al: Supratentorial convexity leptomeningeal schwannoma: case report. Neurosurgery. 51(5):1295-7; discussion 1298, 2002
                                                                          33. Sarma S et al: Nonvestibular schwannomas of the brain: a 7-year experience. Neurosurgery. 50(3):437-48; discussion 438-9, 2002
                                                                          34. Sperfeld AD et al: Occurrence and characterization of peripheral nerve involvement in neurofibromatosis type 2. Brain. 125(Pt 5):996-1004, 2002
                                                                          35. Suh YL et al: Tumors of the central nervous system in Korea: a multicenter study of 3221 cases. J Neurooncol. 56(3):251-9, 2002
                                                                          36. Ture U et al: Infratentorial lateral supracerebellar approach for trochlear nerve schwannoma. J Clin Neurosci. 9(5):595-8, 2002
                                                                          37. Whee SM et al: Intrasellar schwannoma mimicking pituitary adenoma: a case report. J Korean Med Sci. 17(1):147-50, 2002
                                                                          38. Janse AJ et al: Neurofibromatosis type 2 diagnosed in the absence of vestibular schwannomas. A case report and guidelines for a screening protocol for children at risk. Eur J Pediatr. 160(7):439-43, 2001
                                                                          39. Tan TC et al: Subfrontal schwannoma masquerading as meningioma. Singapore Med J. 42(6):275-7, 2001
                                                                          40. Zabel A et al: Management of benign cranial nonacoustic schwannomas by fractionated stereotactic radiotherapy. Int J Cancer. 96(6):356-62, 2001
                                                                          41. Eldevik OP et al: Imaging findings in schwannomas of the jugular foramen. AJNR Am J Neuroradiol. 21(6):1139-44, 2000
                                                                          42. Lingawi SS: Oculomotor nerve schwannoma: MRI appearance. Clin Imaging. 24(2):86-8, 2000
                                                                          43. Woodruff JM et al: Schwannoma. In Kleihues P et al: Tumours of the Nervous System. Lyon: IARC Press. 164-6, 2000