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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
- 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
- Lawson McLean AC et al: Growth dynamics of intramedullary spinal tumors in patients with neurofibromatosis type 2. Clin Neurol Neurosurg. 146:130-7, 2016
- 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
- Maiti TK et al: Spinal meningiomas: clinicoradiological factors predicting recurrence and functional outcome. Neurosurg Focus. 41(2):E6, 2016
- Thomas AK et al: Pediatric schwannomatosis, a rare but distinct form of neurofibromatosis. Pediatr Radiol. 46(3):430-5, 2016
- Lin AL et al: Advances in the treatment of neurofibromatosis-associated tumours. Nat Rev Clin Oncol. 10(11):616-24, 2013
- Asthagiri AR et al: Neurofibromatosis type 2. Lancet. 373(9679):1974-86, 2009
- Evans DG: Neurofibromatosis type 2 (NF2): a clinical and molecular review. Orphanet J Rare Dis. 4:16, 2009
- Holland K et al: Spinal tumors in neurofibromatosis-2: management considerations - a review. J Clin Neurosci. 16(2):169-77, 2009
- 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
- Evans DG et al: Neurofibromatosis type 2. J Med Genet. 37(12):897-904, 2000
- den Bakker MA et al: Expression of the neurofibromatosis type 2 gene in human tissues. J Histochem Cytochem. 47(11):1471-80, 1999
- Kluwe L et al: Identification of NF2 germ-line mutations and comparison with neurofibromatosis 2 phenotypes. Hum Genet. 98(5):534-8, 1996
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References
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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
- 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
- Lawson McLean AC et al: Growth dynamics of intramedullary spinal tumors in patients with neurofibromatosis type 2. Clin Neurol Neurosurg. 146:130-7, 2016
- 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
- Maiti TK et al: Spinal meningiomas: clinicoradiological factors predicting recurrence and functional outcome. Neurosurg Focus. 41(2):E6, 2016
- Thomas AK et al: Pediatric schwannomatosis, a rare but distinct form of neurofibromatosis. Pediatr Radiol. 46(3):430-5, 2016
- Lin AL et al: Advances in the treatment of neurofibromatosis-associated tumours. Nat Rev Clin Oncol. 10(11):616-24, 2013
- Asthagiri AR et al: Neurofibromatosis type 2. Lancet. 373(9679):1974-86, 2009
- Evans DG: Neurofibromatosis type 2 (NF2): a clinical and molecular review. Orphanet J Rare Dis. 4:16, 2009
- Holland K et al: Spinal tumors in neurofibromatosis-2: management considerations - a review. J Clin Neurosci. 16(2):169-77, 2009
- 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
- Evans DG et al: Neurofibromatosis type 2. J Med Genet. 37(12):897-904, 2000
- den Bakker MA et al: Expression of the neurofibromatosis type 2 gene in human tissues. J Histochem Cytochem. 47(11):1471-80, 1999
- Kluwe L et al: Identification of NF2 germ-line mutations and comparison with neurofibromatosis 2 phenotypes. Hum Genet. 98(5):534-8, 1996
