eMedicine Specialties > Radiology > Brain/Spine

Neurofibromatosis Type 2: Multimedia

Author: Andrew L Wagner, MD, Assistant Professor of Radiology, Instructional Faculty, University of Virginia School of Medicine; Director of Neuroradiology, Department of Radiology, Rockingham Memorial Hospital
Contributor Information and Disclosures

Updated: Mar 17, 2008

Multimedia

Axial T1-weighted postcontrast image demonstrates...Media file 1: Axial T1-weighted postcontrast image demonstrates bilateral internal auditory canal-enhancing masses that are diagnostic for neurofibromatosis type 2 (NF2). No biopsy is necessary for the diagnosis. Notice the en plaque meningioma anterior to the brainstem.
Axial T1-weighted postcontrast image demonstrates...

Axial T1-weighted postcontrast image demonstrates bilateral internal auditory canal-enhancing masses that are diagnostic for neurofibromatosis type 2 (NF2). No biopsy is necessary for the diagnosis. Notice the en plaque meningioma anterior to the brainstem.

Sagittal T1-weighted postcontrast image in a pati...Media file 2: Sagittal T1-weighted postcontrast image in a patient with known neurofibromatosis type 2 that demonstrates 2 midline meningiomas (arrowheads), one over the convexity and one along the vein of Galen. The enhancing mass in the medulla (arrow) most likely is an ependymoma.
Sagittal T1-weighted postcontrast image in a pati...

Sagittal T1-weighted postcontrast image in a patient with known neurofibromatosis type 2 that demonstrates 2 midline meningiomas (arrowheads), one over the convexity and one along the vein of Galen. The enhancing mass in the medulla (arrow) most likely is an ependymoma.

Axial postcontrast T1-weighted image demonstrates...Media file 3: Axial postcontrast T1-weighted image demonstrates a large enhancing sellar meningioma surrounding both internal carotid arteries in this case of neurofibromatosis type 2 (NF2) (black arrows). Enhancing tissue in the ethmoid air cells also represents a meningioma extending through the cribriform plate. Meningiomas in patients with NF2 can be more aggressive and invasive than spontaneous meningiomas. Note the small, round, enhancing extra-axial mass posterior to the tectum (white arrow). Although this could represent another meningioma, the shape and location suggest it is a trochlear (CN IV) schwannoma instead.
Axial postcontrast T1-weighted image demonstrates...

Axial postcontrast T1-weighted image demonstrates a large enhancing sellar meningioma surrounding both internal carotid arteries in this case of neurofibromatosis type 2 (NF2) (black arrows). Enhancing tissue in the ethmoid air cells also represents a meningioma extending through the cribriform plate. Meningiomas in patients with NF2 can be more aggressive and invasive than spontaneous meningiomas. Note the small, round, enhancing extra-axial mass posterior to the tectum (white arrow). Although this could represent another meningioma, the shape and location suggest it is a trochlear (CN IV) schwannoma instead.

Sagittal T1-weighted postcontrast image of the th...Media file 4: Sagittal T1-weighted postcontrast image of the thoracic spine demonstrates an enhancing extra-axial meningioma (arrow) anterior to the cord with a complex enhancing mass (arrowheads) that represents a conus ependymoma filling the spinal canal in the lower thoracic spine.
Sagittal T1-weighted postcontrast image of the th...

Sagittal T1-weighted postcontrast image of the thoracic spine demonstrates an enhancing extra-axial meningioma (arrow) anterior to the cord with a complex enhancing mass (arrowheads) that represents a conus ependymoma filling the spinal canal in the lower thoracic spine.

Magnetic resonance image (MRI) in a 62-year-old m...Media file 5: Magnetic resonance image (MRI) in a 62-year-old man who presented with unilateral hearing loss. This MRI demonstrates an enhancing mass in the right internal auditory canal (IAC) that was interpreted as a probable cranial nerve VIII (CN VIII) schwannoma. Although no other abnormalities were detected, the examination was performed as a limited MRI and imaged only the internal IACs. Further screening was not indicated because the chance that this patient had NF2 is exceedingly low.
Magnetic resonance image (MRI) in a 62-year-old m...

Magnetic resonance image (MRI) in a 62-year-old man who presented with unilateral hearing loss. This MRI demonstrates an enhancing mass in the right internal auditory canal (IAC) that was interpreted as a probable cranial nerve VIII (CN VIII) schwannoma. Although no other abnormalities were detected, the examination was performed as a limited MRI and imaged only the internal IACs. Further screening was not indicated because the chance that this patient had NF2 is exceedingly low.

More on Neurofibromatosis Type 2

Overview: Neurofibromatosis Type 2
Imaging: Neurofibromatosis Type 2
Follow-up: Neurofibromatosis Type 2
Multimedia: Neurofibromatosis Type 2
References

References

  1. Baser ME, R Evans DG, Gutmann DH. Neurofibromatosis 2. Curr Opin Neurol. Feb 2003;16(1):27-33. [Medline].

  2. Bance M, Ramsden RT. Management of neurofibromatosis type 2. Ear Nose Throat J. Feb 1999;78(2):91-4, 96. [Medline].

  3. Evans DG. Neurofibromatosis type 2: genetic and clinical features. Ear Nose Throat J. Feb 1999;78(2):97-100. [Medline].

  4. Jacoby LB, MacCollin M, Parry DM, et al. Allelic expression of the NF2 gene in neurofibromatosis 2 and schwannomatosis. Neurogenetics. Apr 1999;2(2):101-8. [Medline].

  5. Fontaine B, Sanson M, Delattre O, et al. Parental origin of chromosome 22 loss in sporadic and NF2 neuromas. Genomics. May 1991;10(1):280-3. [Medline].

  6. Fontaine B, Hanson MP, VonSattel JP, et al. Loss of chromosome 22 alleles in human sporadic spinal schwannomas. Ann Neurol. Feb 1991;29(2):183-6. [Medline].

  7. National Institutes of Health. Consensus Development Conference. Neurofibromatosis Conference Statement. Arch Neurol. 1988;45 (5):575-8. [Medline].

  8. Gutmann DH, Aylsworth A, Carey JC, et al. The diagnostic evaluation and multidisciplinary management of neurofibromatosis 1 and neurofibromatosis 2. JAMA. Jul 2 1997;278(1):51-7. [Medline].

  9. Bosch MM, Boltshauser E, Harpes P, Landau K. Ophthalmologic findings and long-term course in patients with neurofibromatosis type 2. Am J Ophthalmol. Jun 2006;141(6):1068-77. [Medline].

  10. Landau K, Yasargil GM. Ocular fundus in neurofibromatosis type 2. Br J Ophthalmol. Oct 1993;77(10):646-9. [Medline].

  11. Kaye LD, Rothner AD, Beauchamp GR, et al. Ocular findings associated with neurofibromatosis type II. Ophthalmology. Sep 1992;99(9):1424-9. [Medline].

  12. Landau K, Dossetor FM, Hoyt WF, Muci-Mendoza R. Retinal hamartoma in neurofibromatosis 2. Arch Ophthalmol. Mar 1990;108(3):328-9. [Medline].

  13. Smirniotopoulos JG, Murphy FM. The phakomatoses. AJNR Am J Neuroradiol. Mar-Apr 1992;13(2):725-46. [Medline].

  14. Antinheimo J, Sankila R, Carpen O, et al. Population-based analysis of sporadic and type 2 neurofibromatosis-associated meningiomas and schwannomas. Neurology. Jan 11 2000;54(1):71-6. [Medline].

  15. Evans DG, Huson SM, Donnai D, et al. A genetic study of type 2 neurofibromatosis in the United Kingdom. I. Prevalence, mutation rate, fitness, and confirmation of maternal transmission effect on severity. J Med Genet. Dec 1992;29(12):841-6. [Medline].

  16. Baser ME, Evans DG, Jackler RK, et al. Neurofibromatosis 2, radiosurgery and malignant nervous system tumours. Br J Cancer. Feb 2000;82(4):998. [Medline].

  17. Baser ME, Friedman JM, Aeschliman D, et al. Predictors of the risk of mortality in neurofibromatosis 2. Am J Hum Genet. Oct 2002;71(4):715-23. [Medline][Full Text].

  18. Evans DG, Baser ME, O'Reilly B, et al. Management of the patient and family with neurofibromatosis 2: a consensus conference statement. Br J Neurosurg. Feb 2005;19(1):5-12. [Medline].

  19. Baser ME, Evans DG. Lack of sex-ratio distortion in neurofibromatosis 2. Am J Med Genet. Nov 27 2000;95(3):292. [Medline].

  20. MacCollin M, Mautner VF. The diagnosis and management of neurofibromatosis 2 in childhood. Semin Pediatr Neurol. Dec 1998;5(4):243-52. [Medline].

  21. Gillespie JE. Imaging in neurofibromatosis type 2: screening using magnetic resonance imaging. Ear Nose Throat J. Feb 1999;78(2):102-3, 106, 108-9. [Medline].

  22. Mautner VF, Tatagiba M, Lindenau M, et al. Spinal tumors in patients with neurofibromatosis type 2: MR imaging study of frequency, multiplicity, and variety. AJR Am J Roentgenol. Oct 1995;165(4):951-5. [Medline][Full Text].

  23. Egelhoff JC, Bates DJ, Ross JS, et al. Spinal MR findings in neurofibromatosis types 1 and 2. AJNR Am J Neuroradiol. Jul-Aug 1992;13(4):1071-7. [Medline].

  24. Beges C, Revel MP, Gaston A, et al. Trigeminal neuromas: assessment of MRI and CT. Neuroradiology. 1992;34(3):179-83. [Medline].

  25. Allen RW, Harnsberger HR, Shelton C, et al. Low-cost high-resolution fast spin-echo MR of acoustic schwannoma: an alternative to enhanced conventional spin-echo MR?. AJNR Am J Neuroradiol. Aug 1996;17(7):1205-10. [Medline].

  26. Kingsley DP, Brooks GB, Leung AW, Johnson MA. Acoustic neuromas: evaluation by magnetic resonance imaging. AJNR Am J Neuroradiol. Jan-Feb 1985;6(1):1-5. [Medline].

  27. Tien RD, Yang PJ, Chu PK. "Dural tail sign": a specific MR sign for meningioma?. J Comput Assist Tomogr. Jan-Feb 1991;15(1):64-6. [Medline].

  28. Goldsher D, Litt AW, Pinto RS, et al. Dural "tail" associated with meningiomas on Gd-DTPA-enhanced MR images: characteristics, differential diagnostic value, and possible implications for treatment. Radiology. Aug 1990;176(2):447-50. [Medline].

  29. Allcutt DA, Hoffman HJ, Isla A, et al. Acoustic schwannomas in children. Neurosurgery. Jul 1991;29(1):14-8. [Medline].

  30. Aoki S, Barkovich AJ, Nishimura K, et al. Neurofibromatosis types 1 and 2: cranial MR findings. Radiology. Aug 1989;172(2):527-34. [Medline].

  31. Arts WF, Van Dongen KJ. Intracranial calcified deposits in neurofibromatosis. J Neurol Neurosurg Psychiatry. Nov 1986;49(11):1317-20. [Medline].

  32. Baldwin D, King TT, Chevretton E, Morrison AW. Bilateral cerebellopontine angle tumors in neurofibromatosis type 2. J Neurosurg. Jun 1991;74(6):910-5. [Medline].

  33. Celli P, Ferrante L, Acqui M, et al. Neurinoma of the third, fourth, and sixth cranial nerves: a survey and report of a new fourth nerve case. Surg Neurol. Sep 1992;38(3):216-24. [Medline].

  34. Evans DG, Huson SM, Donnai D, et al. A clinical study of type 2 neurofibromatosis. Q J Med. Aug 1992;84(304):603-18. [Medline].

  35. Evans DG, Lye R, Neary W, et al. Probability of bilateral disease in people presenting with a unilateral vestibular schwannoma. J Neurol Neurosurg Psychiatry. Jun 1999;66(6):764-7. [Medline].

  36. Evans DG, Ramsden RT, Shenton A, et al. What are the implications in individuals with unilateral vestibular schwannoma and other neurogenic tumors?. J Neurosurg. Jan 2008;108(1):92-6. [Medline].

  37. Ferner RE. Neurofibromatosis 1 and neurofibromatosis 2: a twenty first century perspective. Lancet Neurol. Apr 2007;6(4):340-51. Review. [Medline].

  38. Halliday AL, Sobel RA, Martuza RL. Benign spinal nerve sheath tumors: their occurrence sporadically and in neurofibromatosis types 1 and 2. J Neurosurg. Feb 1991;74(2):248-53. [Medline].

  39. Hope DG, Mulvihill JJ. Malignancy in neurofibromatosis. Adv Neurol. 1981;29:33-56. [Medline].

  40. Kanter WR, Eldridge R. Maternal effect in central neurofibromatosis. Lancet. Oct 21 1978;2(8095):903. [Medline].

  41. Kim HW, Weinstein SL. Spine update. The management of scoliosis in neurofibromatosis. Spine. Dec 1 1997;22(23):2770-6. [Medline].

  42. King A, Gutmann DH. The question of familial meningiomas and schwannomas: NF2B or not to be?. Neurology. Jan 11 2000;54(1):4-5. [Medline].

  43. Kishore A, O'Reilly BF. A clinical study of vestibular schwannomas in type 2 neurofibromatosis. Clin Otolaryngol Allied Sci. Dec 2000;25(6):561-5. [Medline].

  44. Louw D, Sutherland G, Halliday W, Kaufmann J. Meningiomas mimicking cerebral schwannoma. J Neurosurg. Nov 1990;73(5):715-9. [Medline].

  45. Martuza RL, Eldridge R. Neurofibromatosis 2 (bilateral acoustic neurofibromatosis). N Engl J Med. Mar 17 1988;318(11):684-8. [Medline].

  46. Mayfrank L, Mohadjer M, Wullich B. Intracranial calcified deposits in neurofibromatosis type 2. A CT study of 11 cases. Neuroradiology. 1990;32(1):33-7. [Medline].

  47. Mulvihill JJ, Parry DM, Sherman JL, et al. NIH conference. Neurofibromatosis 1 (Recklinghausen disease) and neurofibromatosis 2 (bilateral acoustic neurofibromatosis). An update. Ann Intern Med. Jul 1 1990;113(1):39-52. [Medline].

  48. Patronas NJ, Courcoutsakis N, Bromley CM, et al. Intramedullary and spinal canal tumors in patients with neurofibromatosis 2: MR imaging findings and correlation with genotype. Radiology. Feb 2001;218(2):434-42. [Medline].

  49. Schefler AC, Dubovy SR, Berrocal AM. Optical coherence tomography characteristics of epiretinal membranes in neurofibromatosis 2. Ophthalmic Surg Lasers Imaging. ;. Jan-Feb 2008;39(1):73-7. [Medline].

  50. Stuckey SL, Harris AJ, Mannolini SM. Detection of acoustic schwannoma: use of constructive interference in the steady state three-dimensional MR. AJNR Am J Neuroradiol. Aug 1996;17(7):1219-25. [Medline].

  51. Wong FL, Boice JD Jr, Abramson DH, et al. Cancer incidence after retinoblastoma. Radiation dose and sarcoma risk. JAMA. Oct 15 1997;278(15):1262-7. [Medline].

  52. Yamakami I, Oishi H, Iwadate Y, Yamaura A. Isolated metastases of adenocarcinoma in the bilateral internal auditory meatuses mimicking neurofibromatosis type 2--case report. Neurol Med Chir (Tokyo). Oct 1999;39(11):756-61. [Medline].

Further Reading

Keywords

neurofibromatosis with bilateral acoustic neuromas, NF2, neurofibromatosis 2, central neurofibromatosis, NF-2, neurofibromatosis II, schwannomatosis, MISME syndrome

Contributor Information and Disclosures

Author

Andrew L Wagner, MD, Assistant Professor of Radiology, Instructional Faculty, University of Virginia School of Medicine; Director of Neuroradiology, Department of Radiology, Rockingham Memorial Hospital
Andrew L Wagner, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, American Society of Neuroradiology, and Radiological Society of North America
Disclosure: Nothing to disclose.

Medical Editor

Mahesh R Patel, MD, Chief of MRI, Department of Radiology, Santa Clara Valley Medical Center
Mahesh R Patel, MD is a member of the following medical societies: Radiological Society of North America
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

Managing Editor

Robert L DeLaPaz, MD, Director, Professor, Department of Radiology, Division of Neuroradiology, Columbia University
Robert L DeLaPaz, MD is a member of the following medical societies: American Society of Neuroradiology, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose.

CME Editor

Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

James G Smirniotopoulos, MD, Professor of Radiology, Neurology, and Biomedical Informatics, Chairman, Department of Radiology and Radiological Sciences, Uniformed Services University of the Health Sciences
James G Smirniotopoulos, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, American Society of Head and Neck Radiology, American Society of Neuroradiology, American Society of Pediatric Neuroradiology, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose.

 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.