Consensus recommendations for current treatments and accelerating clinical trials for patients with neurofibromatosis type 2

American Journal of Medical Genetics, Part A

Volumn 158 A, Issue 1, 2012, Pages 24-41

  Blakeley, Jaishri O ^a   Evans, D Gareth ^b   Adler, John ^c   Brackmann, Derald ^d   Chen, Ruihong ^e   Ferner, Rosalie E ^f   Hanemann, C Oliver ^g   Harris, Gordon ^h   Huson, Susan M ^b   Jacob, Abraham ⁱ   Kalamarides, Michel ^j   Karajannis, Matthias A ^k   Korf, Bruce R ^l   Mautner, Victor Felix ^m   Mcclatchey, Andrea I ^h   Miao, Harry ⁿ   Plotkin, Scott R ^h   Slattery, William ^d   Stemmer Rachamimov, Anat O ^h   Welling, D Bradley ⁱ   Wen, Patrick Y ^o   Widemann, Brigitte ^p   Hunter Schaedle, Kim ^q   Giovannini, Marco ^d   more..

^a JOHNS HOPKINS UNIVERSITY   (United States)

^b UNIVERSITY OF MANCHESTER   (United Kingdom)

^c STANFORD UNIVERSITY   (United States)

^d HOUSE RESEARCH INSTITUTE   (United States)

^e NexGenix Pharmaceuticals   (United States)

^f GUY'S AND ST THOMAS' NHS FOUNDATION TRUST   (United Kingdom)

^g UNIVERSITY OF PLYMOUTH   (United Kingdom)

^h MASSACHUSETTS GENERAL HOSPITAL   (United States)

ⁱ OHIO STATE UNIVERSITY   (United States)

^j BEAUJON HOSPITAL   (France)

^k NEW YORK UNIVERSITY MEDICAL CENTER   (United States)

^l UNIVERSITY OF ALABAMA AT BIRMINGHAM   (United States)

^m UNIVERSITY MEDICAL CENTER HAMBURG EPPENDORF   (Germany)

ⁿ PTC THERAPEUTICS   (United States)

^o DANA FARBER CANCER INSTITUTE   (United States)

^p NATIONAL CANCER INSTITUTE   (United States)

^q Children's Tumor Foundation   (United States)

more..

Author keywords

Ependymoma; Meningioma; Molecular therapy; Neurofibromatosis type 2; Radiotherapy; Schwannoma; Surgery; Vestibular schwannoma

Indexed keywords

ANTINEOPLASTIC AGENT; BEVACIZUMAB; CYTOTOXIC AGENT; EPIDERMAL GROWTH FACTOR; ERLOTINIB; EVEROLIMUS; GEFITINIB; GUANOSINE TRIPHOSPHATE; HEAT SHOCK PROTEIN; LAPATINIB; LIGASE INHIBITOR; MAMMALIAN TARGET OF RAPAMYCIN; NILOTINIB; NXD 30001; PLACEBO; PLATELET DERIVED GROWTH FACTOR; PTC 299; RAPAMYCIN; RAS PROTEIN; SORAFENIB; TEMSIROLIMUS; UNCLASSIFIED DRUG; VASCULOTROPIN;

ACOUSTIC NEURINOMA; ARTICLE; AUTOSOMAL DOMINANT DISORDER; CANCER DIAGNOSIS; CLINICAL EFFECTIVENESS; CLINICAL FEATURE; CLINICAL TRIAL (TOPIC); CONSENSUS; CRANIAL NERVE TUMOR; DISEASE ASSOCIATION; DRUG EFFICACY; DRUG MECHANISM; DRUG SAFETY; DRUG TARGETING; DRUG TOLERABILITY; EARLY DIAGNOSIS; EPENDYMOMA; GLIOBLASTOMA; HEARING LOSS; HISTOPATHOLOGY; HUMAN; HYDROCEPHALUS; IDIOPATHIC DISEASE; INTENSITY MODULATED RADIATION THERAPY; MENINGIOMA; MORBIDITY; MORTALITY; NERVOUS SYSTEM TUMOR; NEURILEMOMA; NEUROFIBROMATOSIS TYPE 2; NEUROSURGERY; NONHUMAN; OVERALL SURVIVAL; PATIENT CARE; PERIPHERAL NERVE TUMOR; PRIORITY JOURNAL; PROTEIN FAMILY; PROTEIN FUNCTION; PROTEIN PROTEIN INTERACTION; PROTEIN TARGETING; QUALITY OF LIFE; RADIATION HAZARD; RADIATION INJURY; RADIOSURGERY; RISK BENEFIT ANALYSIS; SECOND CANCER; SURGICAL APPROACH; SURGICAL TECHNIQUE; SURVIVAL RATE; SYMPTOM; TRANSLATIONAL RESEARCH; TREATMENT OUTCOME; TREATMENT RESPONSE; TUMOR DIAGNOSIS; TUMOR SUPPRESSOR GENE; TUMOR VOLUME; WOUND HEALING IMPAIRMENT;

CLINICAL TRIALS AS TOPIC; CONSENSUS; ENDPOINT DETERMINATION; HUMANS; MENINGIOMA; NEUROFIBROMATOSIS 2; RADIOSURGERY; RESEARCH DESIGN; STANDARD OF CARE;

EID: 84355166399     PISSN: 15524825     EISSN: 15524833     Source Type: Journal    
DOI: 10.1002/ajmg.a.34359     Document Type: Article

References (115)

1. Acebes X, Arruga J, Acebes JJ, Majos C, Muñoz S, Valero IA. 2009. Intracranial meningiomatosis causing foster kennedy syndrome by unilateral optic nerve compression and blockage of the superior sagittal sinus. J Neuroophthalmol 29: 140-142.
2. American Academy of Otolaryngology-Head and Neck Surgery Foundation, INC. 1995. Committee on Hearing and Equilibrium guidelines for the evaluation of hearing preservation in acoustic neuroma (vestibular schwannoma). Otolaryngol Head Neck Surg 113: 179-180.
3. Ammoun S, Cunliffe CH, Allen JC, Chiriboga L, Giancotti FG, Zagzag D, Hanemann CO, Karajannis MA. 2010. ErbB/HER receptor activation and preclinical efficacy of lapatinib in vestibular schwannoma. Neuro Oncol; 12: 834-843.
4. Ammoun S, Hanemann CO. 2011. Emerging therapeutic targets in schwannomas and other merlin-deficient tumors. Nat Rev Neurol; 7: 392-399.
5. Ammoun S, Schmid MC, Triner J, Manley P, Hanemann CO. 2011. Nilotinib alone or in combination with selumetinib is a drug candidate for neurofibromatosis type 2. Neuro Oncol; 13: 759-766.
6. Anderson DE, Leonetti J, Wind JJ, Cribari D, Fahey K. 2005. Resection of large vestibular schwannomas: Facial nerve preservation in the context of surgical approach and patient-assessed outcome. J Neurosurg 102: 643-649.
7. Asthagiri AR, Parry DM, Butman JA, Kim HJ, Tsilou ET, Zhuang Z, Lonser RR. 2009. Neurofibromatosis type 2. Lancet 373: 1974-1986.
8. Balasubramaniam A, Shannon R, Hodaie M, Laperriere N, Michaels H, Guha A. 2007. Glioblastoma multiforme after stereotactic radiotherapy for acoustic neuroma: Case report and review of the literature. Neuro Oncol 9: 447-453.
9. Baser ME, Evans DG, Jackler RK, Sujansky E, Rubenstein A. 2000. Neurofibromatosis 2, radiosurgery and malignant nervous system tumours. Br J Cancer 82: 998.
10. Baser ME, Friedman JM, Aeschliman D, Joe H, Wallace AJ, Ramsden RT, Evans DG. 2002. Predictors of the risk of mortality in neurofibromatosis 2. Am J Hum Genet 71: 715-723.
11. Benesch M, Windelberg M, Sauseng W, Witt V, Fleischhack G, Lackner H, Gadner H, Bode U, Urban C. 2008. Compassionate use of bevacizumab (avastin) in children and young adults with refractory or recurrent solid tumors. Ann Oncol 19: 807-813.
12. Bennett M, Haynes DS. 2007. Surgical approaches and complications in the removal of vestibular schwannomas. Neurosurg Clin N Am 19: 331-343.
13. Blakeley JO, Olson J, Grossman SA, He X, Weingart J, Supko JG, New Approaches to Brain Tumor Therapy (NABTT) Consortium. 2009. Effect of blood brain barrier permeability in recurrent high grade gliomas on the intratumoral pharmacokinetics of methotrexate: A microdialysis study. J Neurooncol 91: 51-58.
14. Blum R, Kloog Y. 2005. Tailoring Ras-pathway inhibitor combinations for cancer therapy. Drug Resist Updat 8: 369-380.
15. Brackmann DE, Green JD Jr. 2008. Translabyrinthine approach for acoustic tumor removal. Neurosurg Clin N Am 19: 251-264.
16. Brackmann DE, Cullen RD, Fisher LM. 2007. Facial nerve function after translabyrinthine vestibular schwannoma surgery. Otolaryngol Head Neck Surg 136: 773-777.
17. Bria C, Wegner RE, Clump DA, Vargo JA, Mintz AH, Heron DE, Burton SA. 2011. Fractionated stereotactic radiosurgery for the treatment of meningiomas. J Cancer Res Ther 7: 52-57.
18. Chen L, Chen LH, Ling F, Liu YS, Samii M, Samii A. 2010. Removal of vestibular schwannoma and facial nerve preservation using small suboccipital retrosigmoid craniotomy. Chin Med J 123: 274-280.
19. Curto M, McClatchey AI. 2008. Nf2/Merlin: A coordinator of receptor signalling and intercellular contact. Br J Cancer 98: 256-262.
20. Curto M, Cole BK, Lallemand D, Liu CH, McClatchey AI. 2007. Contact-dependent inhibition of EGFR signaling by Nf2/Merlin. J Cell Biol 177: 893-903.
21. Day JD, Chen DA, Arriaga M. 2004. Translabyrinthine approach for acoustic neuroma. Neurosurgery 54: 391-395.
22. Doherty JK, Ongkeko W, Crawley B, Andalibi A, Ryan AF. 2008. ErbB and Nrg: Potential molecular targets for vestibular schwannoma pharmacotherapy. Otol Neurotol 29: 50-57.
23. Durand A, Labrousse F, Jouvet A, Bauchet L, Kalamaridès M, Menei P, Deruty R, Moreau JJ, Fèvre-Montange M, Guyotat J. 2009. WHO grade II and III meningiomas: A study of prognostic factors. J Neurooncol 95: 367-375.
24. Evans DG. 2009a. Expression profiling of schwannomas. Abstract presented at 2010 Neurofibromatosis Conference, June 13-16, 2009, Portland, OR.
25. Evans DG. 2009b. Neurofibromatosis type 2 (NF2): A clinical and molecular review. Orphanet J Rare Dis 4: 16-27.
26. Evans DGR, Ramsden R, Birch J. 1999. Paediatric presentation of type 2 neurofibromatosis. Arch Dis Child 81: 496-499.
27. Evans DG, Moran A, King A, Saeed S, Gurusinghe N, Ramsden R. 2005. Incidence of vestibular schwannoma and neurofibromatosis 2 in the North West of England over a 10-year period: Higher incidence than previously thought. Otol Neurotol 26: 93-97.
28. Evans DG, Kalamarides M, Hunter-Schaedle K, Blakeley J, Allen J, Babovic-Vuskanovic D, Belzberg A, Bollag G, Chen R, DiTomaso E, Golfinos J, Harris G, Jacob A, Kalpana G, Karajannis M, Korf B, Kurzrock R, Law M, McClatchey A, Packer R, Roehm P, Rubenstein A, Slattery W 3rd, Tonsgard JH, Welling DB, Widemann B, Yohay K, Giovannini M. 2009. Consensus recommendations to accelerate clinical trials for neurofibromatosis type 2. Clin Cancer Res; 15: 5032-5039.
29. Evans DG, Howard E, Giblin C, Clancy T, Spencer H, Huson SM, Lalloo F. 2010. Birth incidence and prevalence of tumour prone syndromes: Estimates from a UK genetic family register service. Am J Med Genet Part A 152A: 327-332.
30. Food and Drug Administration, Guidance for Industry on Exploratory Investigational New Drug Studies. 2006. Document citation 71 FR 2551; Document number 06-354; pages 2551-2552.
31. Fisher LM, Doherty JK, Lev MH, Slattery WH. 2009. Concordance of bilateral vestibular schwannoma growth and hearing changes in neurofibromatosis 2: Neurofibromatosis 2 natural history consortium. Otol Neurotol 30: 835-841.
32. Fouladi M, Stewart CF, Blaney SM, Onar-Thomas A, Schaiquevich P, Packer RJ, Gajjar A, Kun LE, Boyett JM, Gilbertson RJ. 2010. Phase I trial of lapatinib in children with refractory CNS malignancies: A Pediatric Brain Tumor Consortium study. J Clin Oncol 28: 4221-4227.
33. Friedman RA, Kesser B, Brackmann DE, Fisher LM, Slattery WH, Hitselberger WE. 2003. Long-term hearing preservation after middle fossa removal of vestibular schwannoma. Otolaryngol Head Neck Surg 129: 660-665.
34. Glade Bender JL, Adamson PC, Reid JM, Xu L, Baruchel S, Shaked Y, Kerbel RS, Cooney-Qualter EM, Stempak D, Chen HX, Nelson MD, Krailo MD, Ingle MD, Blaney SM, Kandel JJ, Yamashiro DJ, Children's Oncology Group Study. 2008. Phase I trial and pharmacokinetic study of bevacizumab in pediatric patients with refractory solid tumors: A children's oncology group study. J Clin Oncol 26: 399-405.
35. Goutagny S, Kalamarides M. 2010. Meningiomas and neurofibromatosis. J Neurooncol 99: 341-347.
36. Grayeli AB, Kalamarides M, Bouccara D, Ambert-Dahan E, Sterkers O. 2008. Auditory brainstem implant in neurofibromatosis type 2 and non-neurofibromatosis type 2 patients. Otol Neurotol 29: 1140-1146.
37. Grey PL, Moffat DA, Palmer CR, Hardy DG, Baguley DM. 1996. Factors which influence the facial nerve outcome in vestibular schwannoma surgery. Clin Otolaryngol Allied Sci 21: 409-413.
38. Hadfield KD, Smith MJ, Urquhart JE, Wallace AJ, Bowers NL, King AT, Rutherford SA, Trump D, Newman WG, Evans DG. 2010. Rates of loss of heterozygosity and mitotic recombination in NF2 schwannomas, sporadic vestibular schwannomas and schwannomatosis schwannomas. Oncogene 29: 6216-6221.
39. Halpin C, Rauch SD. 2006. Using audiometric thresholds and word recognition in a treatment study. Otol Neurotol 27: 110-116.
40. Harris GJ, Plotkin SR, MacCollin M, Bhat S, Urban T, Lev MH, Slattery WH. 2008. Three-dimensional volumetrics for tracking vestibular schwannoma growth in neurofibromatosis type II. Neurosurg 62: 1314-1320.
41. Hornigold R, Golding JF, Leschziner G, Vann-Blockley M, Gleeson M, Thomas N, Walsh D, Saeed S, Ferner RE. 2010. The Guy's NF2 impact severity score. Platform presentation. Neurofibromatosis 2-State of the Art, Las Vegas, NV, May 3-4.
42. Inoue Y, Ogawa K, Kanzaki J. 2001. Quality of life of vestibular schwannoma patients after surgery. Acta Otolaryngol 121: 59-61.
43. International RadioSurgery Association. 2004. Brain talk: Acoustic neuroma and patient choice-An educational publication. Volume 9, Number 2. ISSN 1086-427X.
44. James MF, Han S, Polizzano C, Plotkin SR, Manning BD, Stemmer-Rachamimov AO, Gusella JF, Ramesh V. 2009. 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: 4250-4261.
45. Kondziolka D, Flickinger JC, Perez B. 1998. Judicious resection and/or radiosurgery for parasagittal meningiomas: Outcomes from a multicenter review. Gamma Knife Meningioma Study Group. Neurosurgery 43: 405-413.
46. Kondziolka D, Madhok R, Lunsford LD, Mathieu D, Martin JJ, Niranjan A, Flickinger JC. 2009. Stereotactic radiosurgery for convexity meningiomas. J Neurosurg 111: 458-463.
47. Lee JD. 2010. Honokiol, a natural plant product, inhibits the growth of vestibular schwannoma cells. Neurofibromatosis Conference, June 5-8, Baltimore, MD.
48. Lee JY, Niranjan A, McInerney J, Kondziolka D, Flickinger JC, Lunsford LD. 2002. Stereotactic radiosurgery providing long-term tumor control of cavernous sinus meningiomas. J Neurosurg 97: 65-72.
49. Lee TX, Packer MD, Huang J, Akhmametyeva EM, Kulp SK, Chen CS, Giovannini M, Jacob A, Welling DB, Chang LS. 2009. Growth inhibitory and anti-tumour activities of OSU-03012, a novel PDK-1 inhibitor, on vestibular schwannoma and malignant schwannoma cells. Eur J Cancer 45: 1709-1720.
50. Lesinski-Schiedat A, Frohne C, Illg A, Rost U, Matthies C, Battmer RD, Samii M, Lenarz T. 2000. Auditory brainstem implant in auditory rehabilitation of patients with neurofibromatosis type 2: Hannover programme. J Laryngol Otol 27: 15-17.
51. Li W, You L, Cooper J, Schiavon G, Pepe-Caprio A, Zhou L, Ishii R, Giovannini M, Hanemann CO, Long SB, Erdjument-Bromage H, Zhou P, Tempst P, Giancotti FG. 2010. Merlin/NF2 suppresses tumorigenesis by inhibiting the E3 ubiquitin ligase CRL4(DCAF1) in the nucleus. Cell 140: 477-490.
52. Liu AK, Macy ME, Foreman NK. 2009. Bevacizumab as therapy for radiation necrosis in four children with pontine gliomas. Int J Radiat Oncol Biol Phys 75: 1148-1154.
53. López-Lago MA, Okada T, Murillo MM, Socci N, Giancotti FG. 2009. Loss of the tumor suppressor gene NF2, encoding merlin, constitutively activates integrin-dependent mTORC1 signaling. Mol Cell Biol 29: 4235-4249.
54. Louis DN, Scheitauer BW, Budka H, von Deimling A, Kepes JJ. 2000. Meningiomas. In: Kleihues P, Cavenee WK, editors. Pathology and genetics of tumours of the nervous system. Lyon: IARC Press. pp 176-184.
55. Lustig LR, Yeagle J, Driscoll CL, Blevins N, Francis H, Niparko JK. 2006. Cochlear implantation in patients with neurofibromatosis type 2 and bilateral vestibular schwannoma. Otol Neurotol 27: 512-518.
56. Mathieu D, Kondziolka D, Flickinger JC, Niranjan A, Williamson R, Martin JJ, Lunsford LD. 2007. Stereotactic radiosurgery for vestibular schwannomas in patients with neurofibromatosis type 2: An analysis of tumor control, complications, and hearing preservation rates. Neurosurgery 60: 460-468.
57. Mautner VF, Tatagiba M, Guthoff R, Samii M, Pulst SM. 1993. Neurofibromatosis 2 in the pediatric age group. Neurosurgery 33: 92-96.
58. Mautner VF, Lindenau M, Baser ME, Hazim W, Tatagiba M, Haase W, Samii M, Wais R, Pulst SM. 1996. The neuroimaging and clinical spectrum of neurofibromatosis 2. Neurosurgery 38: 880-885, discussion 885-886.
59. Mautner VF, Nguyen R, Kutta H, Fuensterer C, Bokemeyer C, Hagel C, Friedrich RE, Panse J. 2010a. Bevacizumab induces regression of vestibular schwannomas in patients with neurofibromatosis type 2. Neuro Oncol 12: 14-18.
60. Mautner V-F, Nguyen R, Knecht R, Bokemeyer C. 2010b. Radiographic regression of vestibular schwannomas induced by bevacizumab treatment: Sustain under continuous drug application and rebound after drug discontinuation. Ann Oncol 21: 2294-2295.
61. McClatchey AI, Giovannini M. 2005. Membrane organization and tumorigenesis-the NF2 tumor suppressor, Merlin. Genes Dev 19: 2265-2277.
62. McClatchey AI, Fehon RG. 2009. Merlin and the ERM proteins-regulators of receptor distribution and signaling at the cell cortex. Trends Cell Biol; 19: 198-206.
63. Modak S, Cheung NK. 2010. Neuroblastoma: Therapeutic strategies for a clinical enigma. Cancer Treat Rev 36: 307-317.
64. Morrison H, Sperka T, Manent J, Giovannini M, Ponta H, Herrlich P. 2007. Merlin/neurofibromatosis type 2 suppresses growth by inhibiting the activation of Ras and Rac. Cancer Res 67: 520-527.
65. Murgo AJ, Kummar S, Rubinstein L, Gutierrez M, Collins J, Kinders R, Parchment RE, Ji J, Steinberg SM, Yang SX, Hollingshead M, Chen A, Helman L, Wiltrout R, Tomaszewski JE, Doroshow JH. 2008. Designing phase 0 cancer clinical trials. Clin Cancer Res 14: 3675-3682.
66. Myrseth E, Møller P, Pedersen PH, Lund-Johansen M. 2009. Vestibular schwannoma: Surgery or gamma knife radiosurgery? A prospective, nonrandomized study. Neurosurgery 64: 654-661.
67. Nachtegaal J, Smit JH, Smits C, Bezemer PD, van Beek JH, Festen JM, Kramer SE. 2009. The association between hearing status and psychosocial health before the age of 70 years: Results from an internet-based national survey on hearing. Ear Hear 30: 302-312.
68. Nakamura M, Roser F, Michel J, Jacobs C, Samii M. 2003. The natural history of incidental meningiomas. Neurosurgery 53: 62-70.
69. Neary WJ, Hillier VF, Flute T, Stephens D, Ramsden RT, Evans DG. 2010a. Use of a closed set questionnaire to measure primary and secondary effects of neurofibromatosis type 2. J Laryngol Otol 124: 720-728.
70. Neary WJ, Hillier VF, Flute T, Stephens SD, Ramsden RT, Evans DG. 2010b. The relationship between patients' perception of the effects of neurofibromatosis type 2 and the domains of the Short Form-36. Clin Otolaryngol 35: 291-299.
71. Neff BA, Wiet RM, Lasak JM, Ramsden R, Cohen N, Welling DB. 2007. Cochlear implantation in the Neurofibromatosis type 2 patient: Long-term follow-up. Laryngoscope 117: 1069-1072.
72. Nonaka M, Hashimoto Y, Takeshima SN, Aida Y. 2009. The human immunodeficiency virus type 1 Vpr protein and its carboxy-terminally truncated form induce apoptosis in tumor cells. Cancer Cell Int 9: 20-30.
73. Nunes F, MacCollin M. 2003. Neurofibromatosis 2 in the pediatric population. J Child Neurol 18: 718-724.
74. Okada T, You L, Giancotti FG. 2007. Shedding light on Merlin's wizardry. Trends Cell Biol 17: 222-229.
75. Packer RJ, Jakacki R, Horn M, Rood B, Vezina G, MacDonald T, Fisher MJ, Cohen B. 2009. Objective response of multiply recurrent low-grade gliomas to bevacizumab and irinotecan. Pediatr Blood Cancer 52: 791-795.
76. Peyssonnaux C, Eychène A. 2001. The Raf/MEK/ERK pathway: New concepts of activation. Biol Cell 93: 53-62.
77. Pietras K, Pahler J, Bergers G, Hanahan D. 2008. Functions of paracrine PDGF signaling in the proangiogenic tumor stroma revealed by pharmacological targeting. PLoS Med 5: 0123-0138.
78. Plotkin SR, Halpin C, Blakeley JO, Slattery WH III, Welling DB, Chang SM, Loeffler JS, Harris GJ, Sorensen AG, McKenna MJ, Barker FG II. 2009a. Suggested response criteria for Phase II antitumor drug studies for neurofibromatosis type 2 related VS. J Neurooncol 93: 61-77.
79. Plotkin SR, Stemmer-Rachamimov AO, Barker FG II, Halpin C, Padera TP, Tyrrell A, Sorensen AG, Jain RK, di Tomaso E. 2009b. Hearing improvement after bevacizumab in patients with neurofibromatosis type 2. N Engl J Med 361: 358-367.
80. Plotkin SR, Halpin C, McKenna MJ, Loeffler JS, Batchelor TT, Barker FG II. 2010. Erlotinib for progressive vestibular schwannoma in neurofibromatosis 2 patients. Otol Neurotol 31: 1135-1143.
81. Rauch SD. 2008. Clinical practice. Idiopathic sudden sensorineural hearing loss. N Engl J Med 359: 833-840.
82. Raza SM, Gallia GL, Brem H, Weingart JD, Long DM, Olivi A. 2010. Perioperative and long-term outcomes from the management of parasagittal meningiomas invading the superior sagittal sinus. Neurosurgery 67: 885-893.
83. Rouleau GA, Merel P, Lutchman M, Sanson M, Zucman J, Marineau C, Hoang-Xuan K, Demczuk S, Desmaze C, Plougastel B, Pulst SM, Lenoir G, Bijlsma E, Fashold R, Dumanski J, De jong P, Parry D, Eldrige R, Aurias A, Delattre O, Thomas G. 1993. Alteration in a new gene encoding a putative membrane-organizing protein causes neuro-fibromatosis type 2. Nature 363: 515-521.
84. Rowe JG, Radatz MW, Walton L, Hampshire A, Seaman S, Kemeny AA. 2003. Gamma knife stereotactic radiosurgery for unilateral acoustic neuromas. J Neurol Neurosurg Psychiatry 74: 1536-1542.
85. Rowe J, Radatz M, Kemeny A. 2008. Radiosurgery for type II neurofibromatosis. Prog Neurol Surg 21: 176-182.
86. Ruggieri M, Iannetti P, Polizzi A, La Mantia I, Spalice A, Giliberto O, Platania N, Gabriele AL, Albanese V, Pavone L. 2005. Earliest clinical manifestations and natural history of neurofibromatosis type 2 (NF2) in childhood: A study of 24 patients. Neuropediatrics 36: 21-34.
87. Samii M, Matthies C. 1995. Hearing preservation in acoustic tumour surgery. Adv Tech Stand Neurosurg 22: 343-373.
88. Samii M, Matthies C. 1997. Management of 1000 vestibular schwannomas (acoustic neuromas): Surgical management and results with an emphasis on complications and how to avoid them. Neurosurgery 40: 11-21.
89. Samii M, Matthies C, Tatagiba M. 1997. Management of vestibular schwannomas (acoustic neuromas): Auditory and facial nerve function after resection of 120 vestibular schwannomas in patients with neurofibromatosis 2. Neurosurgery 40: 696-705.
90. Samii M, Gerganov V, Samii A. 2008. Microsurgery management of vestibular schwannomas in neurofibromatosis type 2: Indications and results. Prog Neurol Surg 21: 169-175.
91. Schwartz MS, Otto SR, Shannon RV, Hitselberger WE, Brackmann DE. 2008. Auditory brainstem implants. Neurotherapeutics 5: 128-136.
92. Sindou MP, Alvernia JE. 2006. Results of attempted radical tumor removal and venous repair in 100 consecutive meningiomas involving the major dural sinuses. J Neurosurg 105: 514-525.
93. Slattery WH III, Fisher LM, Iqbal Z, Oppenhiemer M. 2004. Vestibular schwannoma growth rates in neurofibromatosis type 2 natural history consortium subjects. Otol Neurotol 25: 811-817.
94. Slattery WH III, Fisher LM, Hitselberger W, Friedman RA, Brackmann DE. 2007. Hearing preservation surgery for neurofibromatosis Type 2-related vestibular schwannoma in pediatric patients. J Neurosurg 106: 255-260.
95. Slattery WH, Hoa M, Bonne N, Friedman RA, Schwartz MS, Fisher LM, Brackmann DE. 2011. Middle fossa decompression for hearing preservation: A review of institutional results and indications. Otol Neurotol 32: 1017-1024.
96. Sorensen AG, Patel S, Harmath C, Bridges S, Synnott J, Sievers A, Yoon YH, Lee EJ, Yang MC, Lewis RF, Harris GJ, Lev MH, Schaefer PW, Buchbinder BR, Barest G, Yamada K, Ponzo J, Kwon HY, Gennete J, Farkas J, Tievsky AL, Ziegler RB, Salhus MRC, Weisskoff R. 2001. Comparison of diameter and perimeter methods for tumor volume calculation. J Clin Oncol 19: 551-557.
97. Stamenkovic I, Yu Q. 2010. Merlin, a "magic" linker between extracellular cues and intracellular signaling pathways that regulate cell motility, proliferation, and survival. Curr Protein Pept Sci 11: 471-484.
98. Stemmer-Rachamimov AO, Ino Y, Lim ZY, Jacoby LB, MacCollin M, Gusella JF, Ramesh V, Louis DN. 1998. Loss of the NF2 gene and merlin occur by the tumorlet stage of schwannoma development in neurofibromatosis 2. J Neuropathol Exp Neurol 57: 1164-1167.
99. Szitkar B. 2010. A meningioma exclusively located inside the superior sagittal sinus responsible for intracranial hypertension. AJNR Am J Neuroradiol 31: E57-E58.
100. Tan DS, Thomas GV, Garrett MD, Banerji U, de Bono JS, Kaye SB, Workman P. 2009. Biomarker-driven early clinical trials in oncology: A paradigm shift in drug development. Cancer J 15: 406-420.
101. Therasse P, Arbuck SG, Eisenhauer EA, Wanders J, Kaplan RS, Rubinstein L, Verweij J, Van Glabbeke M, van Oosterom AT, Christian MC, Gwyther SG. 2000. New guidelines to evaluate the response to treatment in solid tumors. J Nat Cancer Inst 92: 205-216.
102. Thomsen J, Tos M, Møller H, Charabi S. 1994. The choice of approach in surgery for acoustic neuromas (vestibular schwannomas). Tokai J Exp Clin Med 19: 93-101.
103. Thornton AR, Raffin MJ. 1978. Speech-discrimination scores modeled as a binomial variable. J Speech Hear Res 21: 507-518.
104. Tran Ba Huy P, Kania R, Frachet B, Poncet C, Legac MS. 2009. Auditory rehabilitation with cochlear implantation in patients with neurofibromatosis type 2. Acta Otolaryngol 129: 971-975.
105. Trofatter JA, MacCollin MM, Rutter JL, Murrell JR, Duyao MP, Parry DM, Eldridge R, Kley N, Menon AG, Pulaski K, Haase VH, Ambrose CM, Munroe D, Bove C, Haines JL, Martuza JL, MacDonald ME, Seizinger BR, Short MP, Buckler AJ, Gusella JF. 1993. A novel moesin-, ezrin-, radixin-like gene is a candidate for the neurofibromatosis 2 tumor suppressor. Cell 72: 791-800.
106. Urban T, Harris GJ, Barish M, Oliveria G, Zonderman R, Hanlon W, Van den Abbeele A. 2010. Benefits of Utilizing image analysts for radioligical measurements in oncology clinical trials. Appl Clin Trials 19: 32-36.
107. Vincenti V, Pasanisi E, Guida M, Di Trapani G, Sanna M. 2008. Hearing rehabilitation in neurofibromatosis type 2 patients: Cochlear versus auditory brainstem implantation. Audiol Neurootol 13: 273-280.
108. Welling DB, Slater PW, Thomas RD, McGregor JM, Goodman JE. 1999. The learning curve in vestibular schwannoma surgery. Am J Otol 20: 644-648.
109. Wen PY, Yung WK, Lamborn KR, Norden AD, Cloughesy TF, Abrey LE, Fine HA, Chang SM, Robins HI, Fink K, Deangelis LM, Mehta M, Di Tomaso E, Drappatz J, Kesari S, Ligon KL, Aldape K, Jain RK, Stiles CD, Egorin MJ, Prados MD. 2009. Phase II study of imatinib mesylate for recurrent meningiomas (North American Brain Tumor Consortium Study 01-08). Neuro Oncol 11: 853-860.
110. Wentworth S, Pinn M, Bourland JD, Deguzman AF, Ekstrand K, Ellis TL, Glazier SS, McMullen KP, Munley M, Stieber VW, Tatter SB, Shaw EG. 2009. Clinical experience with radiation therapy in the management of neurofibromatosis-associated central nervous system tumors. Int J Radiat Oncol Biol Phys 73: 208-213.
111. Wiegand DA, Ojemann RG, Fickel V. 1996. Surgical treatment of acoustic neuroma (vestibular schwannoma) in the United States: Report from the Acoustic Neuroma Registry. Laryngoscope 106: 58-66.
112. Willis J, Smith C, Ironside JW, Erridge S, Whittle IR, Everington D. 2005. The accuracy of meningioma grading: A 10-year retrospective audit. Neuropathol Appl Neurobiol 31: 141-149.
113. Wong HK, Lahdenranta J, Kamoun WS, Chan AW, McClatchey AI, Plotkin SR, Jain RK, di Tomaso E. 2010. Anti-vascular endothelial growth factor therapies as a novel therapeutic approach to treating neurofibromatosis-related tumors. Cancer Res 70: 3483-3493.
114. Zhao F, Bai Z, Stephens D. 2008. The relationship between changes in self-rated quality of life after cochlear implantation and changes in individual complaints. Clin Otolaryngol 33: 427-434.
115. Zhu H, Woolfenden S, Bronson R, Jaffer ZM, Barluenga S, Winssinger N, Rubenstein AE, Chen R, Charest A. 2010. The novel Hsp90 inhibitor NXD30001 induces tumor regression in a genetically engineered mouse model of glioblastoma multiforme. Mol Cancer Ther 9: 2618-2626.