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
Yao L et al: Therapy of sporadic and NF2-related vestibular schwannoma. Cancers (Basel). 12(4):835, 2020
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
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
Kaul V et al: Management of vestibular schwannoma (including NF2): facial nerve considerations. Otolaryngol Clin North Am. 51(6):1193-212, 2018
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
Evans DG et al: Bilateral vestibular schwannomas in older patients: NF2 or chance? J Med Genet. 52(6):422-4, 2015
Jethanamest D et al: Conservative management of vestibular schwannoma: Predictors of growth and hearing. Laryngoscope. 125(9):2163-8, 2015
Ferner RE et al: Longitudinal evaluation of quality of life in 288 patients with neurofibromatosis 2. J Neurol. 261(5):963-9, 2014
Giovannini M et al: mTORC1 inhibition delays growth of neurofibromatosis type 2 schwannoma. Neuro Oncol. 16(4):493-504, 2014
Matsuo M et al: Characterization of early onset neurofibromatosis type 2. Brain Dev. 36(2):148-52, 2014
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
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
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
Hanemann CO: Magic but treatable? Tumours due to loss of merlin. Brain. 131(Pt 3):606-15, 2008
Fisher LM et al: Distribution of nonvestibular cranial nerve schwannomas in neurofibromatosis 2. Otol Neurotol. 28(8):1083-90, 2007
Bosch MM et al: Optic nerve sheath meningiomas in patients with neurofibromatosis type 2. Arch Ophthalmol. 124(3):379-85, 2006
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
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
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
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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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
Yao L et al: Therapy of sporadic and NF2-related vestibular schwannoma. Cancers (Basel). 12(4):835, 2020
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
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
Kaul V et al: Management of vestibular schwannoma (including NF2): facial nerve considerations. Otolaryngol Clin North Am. 51(6):1193-212, 2018
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
Evans DG et al: Bilateral vestibular schwannomas in older patients: NF2 or chance? J Med Genet. 52(6):422-4, 2015
Jethanamest D et al: Conservative management of vestibular schwannoma: Predictors of growth and hearing. Laryngoscope. 125(9):2163-8, 2015
Ferner RE et al: Longitudinal evaluation of quality of life in 288 patients with neurofibromatosis 2. J Neurol. 261(5):963-9, 2014
Giovannini M et al: mTORC1 inhibition delays growth of neurofibromatosis type 2 schwannoma. Neuro Oncol. 16(4):493-504, 2014
Matsuo M et al: Characterization of early onset neurofibromatosis type 2. Brain Dev. 36(2):148-52, 2014
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
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
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
Hanemann CO: Magic but treatable? Tumours due to loss of merlin. Brain. 131(Pt 3):606-15, 2008
Fisher LM et al: Distribution of nonvestibular cranial nerve schwannomas in neurofibromatosis 2. Otol Neurotol. 28(8):1083-90, 2007
Bosch MM et al: Optic nerve sheath meningiomas in patients with neurofibromatosis type 2. Arch Ophthalmol. 124(3):379-85, 2006
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
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
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
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
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