Current Management of Large Vestibular Schwannomas for NF2 Patients in a National Reference Center.
Adolescent
Adult
Aged
Antineoplastic Agents, Immunological
/ therapeutic use
Bevacizumab
/ therapeutic use
Clinical Decision-Making
Cohort Studies
Decision Trees
Female
France
Hospitals
Humans
Male
Middle Aged
Neurofibromatosis 2
Neuroma, Acoustic
/ drug therapy
Retrospective Studies
Tumor Burden
Young Adult
Auditory brainstem implant, bevacizumab, cochlear implant, cerebellopontine angle
Journal
The Laryngoscope
ISSN: 1531-4995
Titre abrégé: Laryngoscope
Pays: United States
ID NLM: 8607378
Informations de publication
Date de publication:
01 2021
01 2021
Historique:
received:
02
05
2020
revised:
12
07
2020
accepted:
14
07
2020
pubmed:
4
12
2020
medline:
29
1
2021
entrez:
3
12
2020
Statut:
ppublish
Résumé
Recently, treatment decision making for large vestibular schwannomas (VS) in patients with neurofibromatosis type 2 (NF2) has become increasingly challenging due to the availability of multiple therapeutic options including surgery, bevacizumab (an anti-VEGF), radiosurgery, and observation; and it often remains an arbitrary decision based on local practices without firm recommendations. Our objective is to discuss the multimodal treatment options for Koos IV VS in a national reference center for NF2. Single-institution retrospective cohort study. All NF2 patients with Koos IV VS who visited our center, the National Reference Center for NF2 Rare Disease in Pitié-Salpétrière Hospital of Paris, between January 2016 and December 2018 were included. Clinical charts, radiology, operative reports, and audiograms were reviewed. Among 54 NF2 patients with Koos IV VS (mean maximum extrameatal diameter: 34 mm; range:17-62 mm), 27 were operated on for 28 VS; 21 were treated with bevacizumab; and six were observed. In the surgical group, VS resections were gross total, near-total, subtotal, or partial in 32%, 25%, 32%, and 11%, respectively; and a good (House-Brackmann grades I-II) facial nerve function was achieved in 81.5% at 1 year. Hearing was preserved in 14%, 78%, and 66% of the surgical (n = 7), bevacizumab (n = 9), and observation (n = 3) patients, respectively. All therapeutic options, including surgery and/or bevacizumab and occasionally observation, should be proposed to NF2 patients with large VS in the setting of dedicated centers. A decision-making tree is proposed for Koos IV VS management based on tumor evolution, hearing and clinical status of the patient, and contralateral VS size. 4, case series study, historically controlled study Laryngoscope, 131:E98-E107, 2021.
Substances chimiques
Antineoplastic Agents, Immunological
0
Bevacizumab
2S9ZZM9Q9V
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
E98-E107Informations de copyright
© 2020 American Laryngological, Rhinological and Otological Society Inc, "The Triological Society" and American Laryngological Association (ALA).
Références
Evans DGR. Neurofibromatosis type 2 (NF2): a clinical and molecular review. Orphanet J Rare Dis 2009;4:16.
Evans DG, Howard E, Giblin C, et al. Birth incidence and prevalence of tumor-prone syndromes: estimates from a UK family genetic register service. Am J Med Genet A 2010;152A:327-332.
Dewan R, Pemov A, Kim HJ, et al. Evidence of polyclonality in neurofibromatosis type 2-associated multilobulated vestibular schwannomas. Neuro Oncol 2015;17:566-573.
Stivaros SM, Stemmer-Rachamimov AO, Alston R, et al. Multiple synchronous sites of origin of vestibular schwannomas in neurofibromatosis type 2. J Med Genet 2015;52:557-562.
Peyre M, Goutagny S, Bah A, et al. Conservative management of bilateral vestibular schwannomas in neurofibromatosis type 2 patients: hearing and tumor growth results. Neurosurgery 2013;72:907-914.
Peyre M, Bernardeschi D, Sterkers O, Kalamarides M. Natural history of vestibular schwannomas and hearing loss in NF2 patients. Neurochirurgie 2018;64:342-347.
Moffat DA, Lloyd SKW, Macfarlane R, et al. Outcome of translabyrinthine surgery for vestibular schwannoma in neurofibromatosis type 2. Br J Neurosurg 2013;27:446-453.
Zhao F, Wang B, Yang Z, et al. Surgical treatment of large vestibular schwannomas in patients with neurofibromatosis type 2: outcomes on facial nerve function and hearing preservation. J Neurooncol 2018;138:417-424.
Adib SD, Tatagiba M. Surgical management of collision-tumors between vestibular schwannoma and meningioma in the cerebellopontine angle in patients with neurofibromatosis type 2. Acta Neurochir 2019;161:1157-1163.
Troude L, Boucekine M, Montava M, et al. Does NF2 status impact the results of combined surgery and adjunctive gamma knife surgery for large vestibular schwannomas? Neurosurg Rev 2020;43:1191-1199.
Kruyt IJ, Verheul JB, Hanssens PEJ, Kunst HPM. Gamma knife radiosurgery for treatment of growing vestibular schwannomas in patients with neurofibromatosis type 2: a matched cohort study with sporadic vestibular schwannomas. J Neurosurg 2018;128:49-59.
Frischer JM, Gruber E, Schöffmann V, et al. Long-term outcome after gamma knife radiosurgery for acoustic neuroma of all Koos grades: a single-center study. J Neurosurg 2019;130:388-397.
Slattery WH, Fisher LM, Hitselberger W, Friedman RA, Brackmann DE. Hearing preservation surgery for neurofibromatosis type 2-related vestibular schwannoma in pediatric patients. J Neurosurg Pediatr 2007;106:255-260.
Ramsden RT, Freeman SRM, Lloyd SKW, et al. Auditory brainstem implantation in neurofibromatosis type 2: experience from the Manchester programme. Otol Neurotol 2016;37:1267-1274.
Grayeli AB, Kalamarides M, Bouccara D, Ambert-Dahan E, Sterkers O. Auditory brainstem implant in neurofibromatosis type 2 and non-neurofibromatosis type 2 patients. Otol Neurotol 2008;29:1140-1146.
Jia H, El Sayed MME, Smail M, et al. Neurofibromatosis type 2: hearing preservation and rehabilitation. Neurochirurgie 2018;64:348-354.
Plotkin SR, Stemmer-Rachamimov AO, Barker FG, et al. Hearing improvement after bevacizumab in patients with neurofibromatosis type 2. N Engl J Med 2009;361:358-367.
Plotkin SR, Merker VL, Halpin C, Jennings D. Bevacizumab for progressive vestibular schwannoma in neurofibromatosis type 2: a retrospective review of 31 patients. Otol Neurotol 2012;33:1046-1052.
Goutagny S, Kalamarides M. Medical treatment in neurofibromatosis type 2. Review of the literature and presentation of clinical reports. Neurochirurgie 2018;64:370-374.
Morris KA, Golding JF, Axon PR, et al. Bevacizumab in neurofibromatosis type 2 (NF2) related vestibular schwannomas: a nationally coordinated approach to delivery and prospective evaluation. Neurooncol Pract 2016;3:281-289.
Mautner V-F, Nguyen R, Kutta H, et al. Bevacizumab induces regression of vestibular schwannomas in patients with neurofibromatosis type 2. Neuro Oncol 2010;12:14-18.
Alanin MC, Klausen C, Caye-Thomasen P, et al. The effect of bevacizumab on vestibular schwannoma tumour size and hearing in patients with neurofibromatosis type 2. Eur Arch Otorhinolaryngol 2015;272:3627-3633.
Gugel I, Kluwe L, Zipfel J, et al. Minimal effect of bevacizumab treatment on residual vestibular schwannomas after partial resection in young neurofibromatosis type 2 patients. Cancer 2019;11:1862.
Lu VM, Ravindran K, Graffeo CS, et al. Efficacy and safety of bevacizumab for vestibular schwannoma in neurofibromatosis type 2: a systematic review and meta-analysis of treatment outcomes. J Neurooncol 2019;144:239-248.
Plotkin SR, Duda DG, Muzikansky A, et al. Multicenter, prospective, phase II and biomarker study of high-dose bevacizumab as induction therapy in patients with neurofibromatosis type 2 and progressive vestibular schwannoma. J Clin Oncol 2019;37:3446-3454.
Erickson NJ, Schmalz PGR, Agee BS, et al. Koos classification of vestibular schwannomas: a reliability study. Neurosurgery 2019;85:409-414.
Halliday D, Emmanouil B, Pretorius P, et al. Genetic severity score predicts clinical phenotype in NF2. J Med Genet 2017;54:657-664.
Daoudi H, Lahlou G, Degos V, Sterkers O, Nguyen Y, Kalamarides M. Improving facial nerve outcome and hearing preservation by different degrees of vestibular schwannoma resection guided by intraoperative facial nerve electromyography. Acta Neurochir 2020;162:1983-1993.
Committee on Hearing and Equilibrium guidelines for the evaluation of hearing preservation in acoustic neuroma (vestibular schwannoma). Otolaryngol Neck Surg 2004;130:20A-20A.
House JW, Brackmann DE. Facial nerve grading system. Otolaryngol Head Neck Surg 1985;93:146-147.
Bernardeschi D, Pyatigorskaya N, Vanier A, et al. Role of electrophysiology in guiding near-total resection for preservation of facial nerve function in the surgical treatment of large vestibular schwannomas. J Neurosurg 2018;128:903-910.
Jacob JT, Carlson ML, Driscoll CL, Link MJ. Volumetric analysis of tumor control following subtotal and near-total resection of vestibular schwannoma. Laryngoscope 2016;126:1877-1882.
Plotkin SR, Halpin C, Blakeley JO, et al. Suggested response criteria for phase II antitumor drug studies for neurofibromatosis type 2 related vestibular schwannoma. J Neurooncol 2009;93:61-77.
Gurgel RK, Jackler RK, Dobie RA, Popelka GR. A new standardized format for reporting hearing outcome in clinical trials. Otolaryngol Neck Surg 2012;147:803-807.
Torres R, Nguyen Y, Vanier A, et al. Multivariate analysis of factors influencing facial nerve outcome following microsurgical resection of vestibular schwannoma. Otolaryngol Neck Surg 2017;156:525-533.
Bernat I, Grayeli AB, Esquia G, Zhang Z, Kalamarides M, Sterkers O. Intraoperative electromyography and surgical observations as predictive factors of facial nerve outcome in vestibular schwannoma surgery. Otol Neurotol 2010;31:306-312.
Gugel I, Grimm F, Teuber C, et al. Management of NF2-associated vestibular schwannomas in children and young adults: influence of surgery and clinical factors on tumor volume and growth rate. J Neurosurg Pediatr 2019;23:1-9.
Peyre M, Goutagny S, Imbeaud S, et al. Increased growth rate of vestibular schwannoma after resection of contralateral tumor in neurofibromatosis type 2. Neuro Oncol 2011;13:1125-1132.
Goutagny S, Raymond E, Esposito-Farese M, et al. Phase II study of mTORC1 inhibition by everolimus in neurofibromatosis type 2 patients with growing vestibular schwannomas. J Neurooncol 2015;122:313-320.