{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,12]],"date-time":"2026-06-12T10:23:07Z","timestamp":1781259787767,"version":"3.54.1"},"reference-count":56,"publisher":"Springer Science and Business Media LLC","issue":"10","license":[{"start":{"date-parts":[[2020,6,16]],"date-time":"2020-06-16T00:00:00Z","timestamp":1592265600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2020,6,16]],"date-time":"2020-06-16T00:00:00Z","timestamp":1592265600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100009397","name":"Medizinischen Fakult\u00e4t, Eberhard Karls Universit\u00e4t T\u00fcbingen","doi-asserted-by":"publisher","award":["2565-0-0"],"award-info":[{"award-number":["2565-0-0"]}],"id":[{"id":"10.13039\/501100009397","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Childs Nerv Syst"],"published-print":{"date-parts":[[2020,10]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>We reviewed our experience in managing of NF2-associated vestibular schwannoma (VS) in children and young adults regarding the effect of surgery and postoperative bevacizumab treatment. A total of 579 volumetric and hearing data sets were analyzed. The effect of surgery on tumor volume and growth rate was investigated in 46 tumors and on hearing function in 39 tumors. Long-term hearing follow-up behavior was compared with 20 non-operated ears in additional 15 patients. Sixteen operated VS were treated with bevacizumab. Mutation analysis of the NF2 gene was performed in 25 patients. Surgery significantly slowed down VS growth rate. Factors associated with a higher growth rate were increasing patient age, tumor volume, and constitutional truncating mutations. Immediately after surgery, functional hearing was maintained in 82% of ears. Deterioration of hearing was associated with initial hearing quality, larger tumor volumes, and larger resection amounts. Average hearing scores were initially better in the group of non-operated VS. Over time, hearing scores in both groups worsened with a similar dynamic. During bevacizumab treatment of residual tumors, four different patterns of growth were observed. Decompression of the internal auditory canal with various degrees of tumor resection decreases the postoperative tumor growth rates. Carefully tailored BAEP-guided surgery does not cause additional hearing deterioration. Secondary bevacizumab treatment showed heterogenous effects both regarding tumor size and hearing preservation. It seems that postoperative tumor residuals, that grow slower, behave differently to bevacizumab than reported for not-operated faster growing VS.<\/jats:p>","DOI":"10.1007\/s00381-020-04728-x","type":"journal-article","created":{"date-parts":[[2020,6,16]],"date-time":"2020-06-16T17:02:50Z","timestamp":1592326970000},"page":"2471-2480","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Managing NF2-associated vestibular schwannomas in children and young adults: review of an institutional series regarding effects of surgery and bevacizumab on growth rates, tumor volume, and hearing quality"],"prefix":"10.1007","volume":"36","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2923-5949","authenticated-orcid":false,"given":"Isabel","family":"Gugel","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julian","family":"Zipfel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philip","family":"Hartjen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lan","family":"Kluwe","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcos","family":"Tatagiba","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Victor-Felix","family":"Mautner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin Ulrich","family":"Schuhmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2020,6,16]]},"reference":[{"key":"4728_CR1","doi-asserted-by":"crossref","unstructured":"Trofatter JA, MacCollin MM, Rutter JL, Murrell JR, Duyao MP, Parry DM, Eldridge R, Kley N, Menon AG, Pulaski K et al (1993) A novel moesin-, ezrin-, radixin-like gene is a candidate for the neurofibromatosis 2 tumor suppressor. Cell 75(4):826","DOI":"10.1016\/0092-8674(93)90406-G"},{"key":"4728_CR2","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1097\/00129492-200501000-00016","volume":"26","author":"DG Evans","year":"2005","unstructured":"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\u201397","journal-title":"Otol Neurotol"},{"key":"4728_CR3","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1002\/ajmg.a.33139","volume":"152A","author":"D Evans","year":"2009","unstructured":"Evans D, Howard E, Giblin C, Clancy T, Spencer H, Huson S, Lalloo F (2009) Birth incidence and prevalence of tumour prone syndromes: estimates from UK genetic family register service. Am J Med Genet 152A:327\u2013332","journal-title":"Am J Med Genet"},{"key":"4728_CR4","doi-asserted-by":"crossref","first-page":"1974","DOI":"10.1016\/S0140-6736(09)60259-2","volume":"373","author":"AR Asthagiri","year":"2009","unstructured":"Asthagiri AR, Parry DM, Butman JA, Kim HJ, Tsilou ET, Zhuang Z, Lonser RR (2009) Neurofibromatosis type 2. Lancet 373:1974\u20131986","journal-title":"Lancet"},{"key":"4728_CR5","doi-asserted-by":"crossref","first-page":"576","DOI":"10.1097\/GIM.0b013e318211faa9","volume":"13","author":"ME Baser","year":"2011","unstructured":"Baser ME, Friedman JM, Joe H, Shenton A, Wallace AJ, Ramsden RT, Evans DG (2011) Empirical development of improved diagnostic criteria for neurofibromatosis 2. Genet Med 13:576\u2013581","journal-title":"Genet Med"},{"key":"4728_CR6","doi-asserted-by":"crossref","first-page":"450","DOI":"10.1002\/ajmg.1320520411","volume":"52","author":"DM Parry","year":"1994","unstructured":"Parry DM, Eldridge R, Kaiser-Kupfer MI, Bouzas EA, Pikus A, Patronas N (1994) Neurofibromatosis 2 (NF2): clinical characteristics of 63 affected individuals and clinical evidence for heterogeneity. Am J Med Genet 52:450\u2013461","journal-title":"Am J Med Genet"},{"key":"4728_CR7","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1097\/01.mao.0000217356.73463.e7","volume":"27","author":"SE Stangerup","year":"2006","unstructured":"Stangerup SE, Caye-Thomasen P, Tos M, Thomsen J (2006) The natural history of vestibular schwannoma. Otol Neurotol 27:547\u2013552","journal-title":"Otol Neurotol"},{"key":"4728_CR8","doi-asserted-by":"crossref","first-page":"776","DOI":"10.1097\/00005072-199511000-00004","volume":"54","author":"J Antinheimo","year":"1995","unstructured":"Antinheimo J, Haapasalo H, Seppala M, Sainio M, Carpen O, Jaaskelainen J (1995) Proliferative potential of sporadic and neurofibromatosis 2-associated schwannomas as studied by MIB-1 (Ki-67) and PCNA labeling. J Neuropathol Exp Neurol 54:776\u2013782","journal-title":"J Neuropathol Exp Neurol"},{"key":"4728_CR9","first-page":"30","volume":"22","author":"K Saito","year":"2003","unstructured":"Saito K, Kato M, Susaki N, Nagatani T, Nagasaka T, Yoshida J (2003) Expression of Ki-67 antigen and vascular endothelial growth factor in sporadic and neurofibromatosis type 2-associated schwannomas. Clin Neuropathol 22:30\u201334","journal-title":"Clin Neuropathol"},{"key":"4728_CR10","doi-asserted-by":"crossref","first-page":"106","DOI":"10.1097\/00005072-199303000-00002","volume":"52","author":"RA Sobel","year":"1993","unstructured":"Sobel RA (1993) Vestibular (acoustic) schwannomas: histologic features in neurofibromatosis 2 and in unilateral cases. J Neuropathol Exp Neurol 52:106\u2013113","journal-title":"J Neuropathol Exp Neurol"},{"key":"4728_CR11","doi-asserted-by":"crossref","first-page":"450","DOI":"10.1136\/jmg.35.6.450","volume":"35","author":"DG Evans","year":"1998","unstructured":"Evans DG, Trueman L, Wallace A, Collins S, Strachan T (1998) Genotype\/phenotype correlations in type 2 neurofibromatosis (NF2): evidence for more severe disease associated with truncating mutations. J Med Genet 35:450\u2013455","journal-title":"J Med Genet"},{"key":"4728_CR12","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1002\/(SICI)1096-8628(19980518)77:3<228::AID-AJMG8>3.0.CO;2-L","volume":"77","author":"L Kluwe","year":"1998","unstructured":"Kluwe L, MacCollin M, Tatagiba M, Thomas S, Hazim W, Haase W, Mautner VF (1998) Phenotypic variability associated with 14 splice-site mutations in the NF2 gene. Am J Med Genet 77:228\u2013233","journal-title":"Am J Med Genet"},{"key":"4728_CR13","doi-asserted-by":"crossref","first-page":"534","DOI":"10.1007\/s004390050255","volume":"98","author":"L Kluwe","year":"1996","unstructured":"Kluwe L, Bayer S, Baser ME, Hazim W, Haase W, Funsterer C, Mautner VF (1996) Identification of NF2 germ-line mutations and comparison with neurofibromatosis 2 phenotypes. Hum Genet 98:534\u2013538","journal-title":"Hum Genet"},{"key":"4728_CR14","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1159\/000156905","volume":"21","author":"M Samii","year":"2008","unstructured":"Samii M, Gerganov V, Samii A (2008) Microsurgery management of vestibular schwannomas in neurofibromatosis type 2: indications and results. Prog Neurol Surg 21:169\u2013175","journal-title":"Prog Neurol Surg"},{"key":"4728_CR15","doi-asserted-by":"crossref","first-page":"358","DOI":"10.1056\/NEJMoa0902579","volume":"361","author":"SR Plotkin","year":"2009","unstructured":"Plotkin SR, Stemmer-Rachamimov AO, Barker FG 2nd, Halpin C, Padera TP, Tyrrell A, Sorensen AG, Jain RK, di Tomaso E (2009) Hearing improvement after bevacizumab in patients with neurofibromatosis type 2. N Engl J Med 361:358\u2013367","journal-title":"N Engl J Med"},{"key":"4728_CR16","doi-asserted-by":"crossref","first-page":"1046","DOI":"10.1097\/MAO.0b013e31825e73f5","volume":"33","author":"SR Plotkin","year":"2012","unstructured":"Plotkin SR, Merker VL, Halpin C, Jennings D, McKenna MJ, Harris GJ, Barker FG 2nd (2012) Bevacizumab for progressive vestibular schwannoma in neurofibromatosis type 2: a retrospective review of 31 patients. Otol Neurotol 33:1046\u20131052","journal-title":"Otol Neurotol"},{"key":"4728_CR17","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1002\/ajmg.a.34359","volume":"158A","author":"JO Blakeley","year":"2012","unstructured":"Blakeley JO, Evans DG, Adler J, Brackmann D, Chen R, Ferner RE, Hanemann CO, Harris G, Huson SM, Jacob A, Kalamarides M, Karajannis MA, Korf BR, Mautner VF, McClatchey AI, Miao H, Plotkin SR, Slattery W 3rd, Stemmer-Rachamimov AO, Welling DB, Wen PY, Widemann B, Hunter-Schaedle K, Giovannini M (2012) Consensus recommendations for current treatments and accelerating clinical trials for patients with neurofibromatosis type 2. Am J Med Genet A 158A:24\u201341","journal-title":"Am J Med Genet A"},{"key":"4728_CR18","doi-asserted-by":"crossref","first-page":"696","DOI":"10.1097\/00006123-199704000-00007","volume":"40","author":"M Samii","year":"1997","unstructured":"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\u2013705 discussion 705-696","journal-title":"Neurosurgery"},{"key":"4728_CR19","doi-asserted-by":"crossref","first-page":"919","DOI":"10.1097\/00006123-199705000-00007","volume":"40","author":"C Matthies","year":"1997","unstructured":"Matthies C, Samii M (1997) Management of vestibular schwannomas (acoustic neuromas): the value of neurophysiology for evaluation and prediction of auditory function in 420 cases. Neurosurgery 40:919\u2013929 discussion 929-930","journal-title":"Neurosurgery"},{"key":"4728_CR20","doi-asserted-by":"crossref","first-page":"2627","DOI":"10.1007\/s00405-013-2753-0","volume":"271","author":"T Mom","year":"2014","unstructured":"Mom T, Montalban A, Khalil T, Gabrillargues J, Chazal J, Gilain L, Avan P (2014) Vasospasm of labyrinthine artery in cerebellopontine angle surgery: evidence brought by distortion-product otoacoustic emissions. Eur Arch Otorhinolaryngol 271:2627\u20132635","journal-title":"Eur Arch Otorhinolaryngol"},{"key":"4728_CR21","doi-asserted-by":"crossref","first-page":"1530","DOI":"10.1097\/MAO.0b013e3182355855","volume":"32","author":"RA Friedman","year":"2011","unstructured":"Friedman RA, Goddard JC, Wilkinson EP, Schwartz MS, Slattery WH 3rd, Fayad JN, Brackmann DE (2011) Hearing preservation with the middle cranial fossa approach for neurofibromatosis type 2. Otol Neurotol 32:1530\u20131537","journal-title":"Otol Neurotol"},{"key":"4728_CR22","doi-asserted-by":"crossref","first-page":"487","DOI":"10.1038\/ncponc1157","volume":"5","author":"SR Plotkin","year":"2008","unstructured":"Plotkin SR, Singh MA, O\u2019Donnell CC, Harris GJ, McClatchey AI, Halpin C (2008) Audiologic and radiographic response of NF2-related vestibular schwannoma to erlotinib therapy. Nat Clin Pract Oncol 5:487\u2013491","journal-title":"Nat Clin Pract Oncol"},{"key":"4728_CR23","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1007\/s11060-015-1828-8","volume":"124","author":"A Hochart","year":"2015","unstructured":"Hochart A, Gaillard V, Baroncini M, Andre N, Vannier JP, Vinchon M, Dubrulle F, Lejeune JP, Vincent C, Neve V, Sudour Bonnange H, Bonne NX, Leblond P (2015) Bevacizumab decreases vestibular schwannomas growth rate in children and teenagers with neurofibromatosis type 2. J Neuro-Oncol 124:229\u2013236","journal-title":"J Neuro-Oncol"},{"key":"4728_CR24","doi-asserted-by":"crossref","unstructured":"Gugel I, Grimm F, Teuber C, Kluwe L, Mautner VF, Tatagiba M, Schuhmann MU (2019) 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: 1-9","DOI":"10.3171\/2019.6.PEDS1947"},{"key":"4728_CR25","doi-asserted-by":"crossref","unstructured":"Gugel I, Grimm F, Liebsch M, Zipfel J, Teuber C, Kluwe L, Mautner VF, Tatagiba M, Schuhmann MU (2019) Impact of surgery on long-term results of hearing in neurofibromatosis type-2 associated vestibular schwannomas. Cancers 11","DOI":"10.3390\/cancers11091376"},{"key":"4728_CR26","doi-asserted-by":"crossref","unstructured":"Gugel I, Kluwe L, Zipfel J, Teuber C, Tatagiba M, Mautner VF, Schuhmann MU, Grimm F (2019) Minimal effect of bevacizumab treatment on residual vestibular schwannomas after partial resection in young neurofibromatosis type 2 patients. Cancers 11","DOI":"10.3390\/cancers11121862"},{"key":"4728_CR27","doi-asserted-by":"crossref","first-page":"813","DOI":"10.1080\/03610927708827533","volume":"6","author":"PW Holland","year":"1977","unstructured":"Holland PW, Welsch RE (1977) Robust regression using iteratively reweighted least-squares. Communications in Statistics - Theory and Methods 6:813\u2013827","journal-title":"Communications in Statistics - Theory and Methods"},{"key":"4728_CR28","doi-asserted-by":"crossref","first-page":"248","DOI":"10.1097\/00006123-199702000-00005","volume":"40","author":"M Samii","year":"1997","unstructured":"Samii M, Matthies C (1997) Management of 1000 vestibular schwannomas (acoustic neuromas): hearing function in 1000 tumor resections. Neurosurgery 40:248\u2013260 discussion 260-242","journal-title":"Neurosurgery"},{"key":"4728_CR29","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1177\/000348948809700110","volume":"97","author":"G Gardner","year":"1988","unstructured":"Gardner G, Robertson JH (1988) Hearing preservation in unilateral acoustic neuroma surgery. Ann Otol Rhinol Laryngol 97:55\u201366","journal-title":"Ann Otol Rhinol Laryngol"},{"key":"4728_CR30","doi-asserted-by":"crossref","unstructured":"(1995) Committee on Hearing and Equilibrium guidelines for the evaluation of hearing preservation in acoustic neuroma (vestibular schwannoma). American Academy of Otolaryngology-Head and Neck Surgery Foundation, INC. Otolaryngol Head Neck Surg 113: 179-180","DOI":"10.1016\/S0194-5998(95)70101-X"},{"key":"4728_CR31","first-page":"274","volume":"49","author":"DE Brackmann","year":"2001","unstructured":"Brackmann DE, Fayad JN, Slattery WH 3rd, Friedman RA, Day JD, Hitselberger WE, Owens RM (2001) Early proactive management of vestibular schwannomas in neurofibromatosis type 2. Neurosurgery 49:274\u2013280 discussion 280-273","journal-title":"Neurosurgery"},{"key":"4728_CR32","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1177\/019459988509300202","volume":"93","author":"JW House","year":"1985","unstructured":"House JW, Brackmann DE (1985) Facial nerve grading system. Otolaryngol Head Neck Surg 93:146\u2013147","journal-title":"Otolaryngol Head Neck Surg"},{"key":"4728_CR33","doi-asserted-by":"crossref","first-page":"217","DOI":"10.3171\/jns.2002.96.2.0217","volume":"96","author":"ME Baser","year":"2002","unstructured":"Baser ME, Makariou EV, Parry DM (2002) Predictors of vestibular schwannoma growth in patients with neurofibromatosis Type 2. J Neurosurg 96:217\u2013222","journal-title":"J Neurosurg"},{"key":"4728_CR34","doi-asserted-by":"crossref","first-page":"2403","DOI":"10.1007\/s00381-016-3257-1","volume":"32","author":"N Bonne","year":"2016","unstructured":"Bonne N, Aboukais R, Baroncini M, Hochart A, Leblond P, Broly F, Dubrulle F, Lejeune JP, Vincent C (2016) Pediatric neurofibromatosis type 2: clinical and molecular presentation, management of vestibular schwannomas, and hearing rehabilitation. Childs Nerv Syst 32:2403\u20132413","journal-title":"Childs Nerv Syst"},{"key":"4728_CR35","doi-asserted-by":"crossref","first-page":"109","DOI":"10.3171\/2012.3.JNS111649","volume":"117","author":"MS Dirks","year":"2012","unstructured":"Dirks MS, Butman JA, Kim HJ, Wu T, Morgan K, Tran AP, Lonser RR, Asthagiri AR (2012) Long-term natural history of neurofibromatosis Type 2-associated intracranial tumors. J Neurosurg 117:109\u2013117","journal-title":"J Neurosurg"},{"key":"4728_CR36","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1007\/s10143-009-0223-3","volume":"32","author":"E Ito","year":"2009","unstructured":"Ito E, Saito K, Yatsuya H, Nagatani T, Otsuka G (2009) Factors predicting growth of vestibular schwannoma in neurofibromatosis type 2. Neurosurg Rev 32:425\u2013433","journal-title":"Neurosurg Rev"},{"key":"4728_CR37","doi-asserted-by":"crossref","first-page":"1983","DOI":"10.1007\/s00701-015-2542-1","volume":"157","author":"H Li","year":"2015","unstructured":"Li H, Hao SY, Wang L, Li D, Wu Z, Zhang LW, Zhang JT (2015) Factors influencing the growth rate of vestibular schwannoma in patients with neurofibromatosis type 2. Acta Neurochir 157:1983\u20131990","journal-title":"Acta Neurochir"},{"key":"4728_CR38","doi-asserted-by":"crossref","first-page":"223","DOI":"10.3171\/jns.2002.96.2.0223","volume":"96","author":"VF Mautner","year":"2002","unstructured":"Mautner VF, Baser ME, Thakkar SD, Feigen UM, Friedman JM, Kluwe L (2002) Vestibular schwannoma growth in patients with neurofibromatosis type 2: a longitudinal study. J Neurosurg 96:223\u2013228","journal-title":"J Neurosurg"},{"key":"4728_CR39","doi-asserted-by":"crossref","unstructured":"Peyre M, Goutagny S, Bah A, Bernardeschi D, Larroque B, Sterkers O, Kalamarides M (2013) Conservative management of bilateral vestibular schwannomas in neurofibromatosis type 2 patients: hearing and tumor growth results. Neurosurgery 72:907\u2013913; discussion 914 quiz 914","DOI":"10.1227\/NEU.0b013e31828bae28"},{"key":"4728_CR40","doi-asserted-by":"crossref","first-page":"1125","DOI":"10.1093\/neuonc\/nor101","volume":"13","author":"M Peyre","year":"2011","unstructured":"Peyre M, Goutagny S, Imbeaud S, Bozorg-Grayeli A, Felce M, Sterkers O, Kalamarides M (2011) Increased growth rate of vestibular schwannoma after resection of contralateral tumor in neurofibromatosis type 2. Neuro-oncology 13:1125\u20131132","journal-title":"Neuro-oncology"},{"key":"4728_CR41","doi-asserted-by":"crossref","first-page":"811","DOI":"10.1097\/00129492-200409000-00027","volume":"25","author":"WH Slattery 3rd","year":"2004","unstructured":"Slattery WH 3rd, Fisher LM, Iqbal Z, Oppenhiemer M (2004) Vestibular schwannoma growth rates in neurofibromatosis type 2 natural history consortium subjects. Otol Neurotol 25:811\u2013817","journal-title":"Otol Neurotol"},{"key":"4728_CR42","doi-asserted-by":"crossref","first-page":"1314","DOI":"10.1227\/01.neu.0000333303.79931.83","volume":"62","author":"GJ Harris","year":"2008","unstructured":"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. Neurosurgery 62:1314\u20131319 discussion 1319-1320","journal-title":"Neurosurgery"},{"key":"4728_CR43","doi-asserted-by":"crossref","first-page":"653","DOI":"10.1016\/j.acra.2015.01.005","volume":"22","author":"B Gaonkar","year":"2015","unstructured":"Gaonkar B, Macyszyn L, Bilello M, Sadaghiani MS, Akbari H, Atthiah MA, Ali ZS, Da X, Zhan Y, O\u2019Rourke D, Grady SM, Davatzikos C (2015) Automated tumor volumetry using computer-aided image segmentation. Acad Radiol 22:653\u2013661","journal-title":"Acad Radiol"},{"key":"4728_CR44","doi-asserted-by":"crossref","first-page":"650","DOI":"10.3171\/2014.3.PEDS13455","volume":"13","author":"JW Choi","year":"2014","unstructured":"Choi JW, Lee JY, Phi JH, Wang KC, Chung HT, Paek SH, Kim DG, Park SH, Kim SK (2014) Clinical course of vestibular schwannoma in pediatric neurofibromatosis Type 2. J Neurosurg Pediatr 13:650\u2013657","journal-title":"J Neurosurg Pediatr"},{"key":"4728_CR45","doi-asserted-by":"crossref","unstructured":"Buhl JL, Selt F, Hielscher T, Guiho R, Ecker J, Sahm F, Ridinger J, Riehl D, Usta D, Ismer B, Sommerkamp AC, Martinez-Babera JP, Wefers AK, Remke M, Picard D, Pusch S, Gronych J, Oehme I, van Tilburg CM, Kool M, Kuhn D, Capper D, von Deimling A, Schuhmann M, Herold-Mende C, Korshunov A, Brummer T, Pfister SM, Jones DTW, Witt O, Milde T (2018) The senescence-associated secretory phenotype mediates oncogene-induced senescence in pediatric pilocytic astrocytoma. Clin Cancer Res","DOI":"10.1158\/1078-0432.CCR-18-1965"},{"key":"4728_CR46","first-page":"529","volume":"59","author":"DM Parry","year":"1996","unstructured":"Parry DM, MacCollin MM, Kaiser-Kupfer MI, Pulaski K, Nicholson HS, Bolesta M, Eldridge R, Gusella JF (1996) Germ-line mutations in the neurofibromatosis 2 gene: correlations with disease severity and retinal abnormalities. Am J Hum Genet 59:529\u2013539","journal-title":"Am J Hum Genet"},{"key":"4728_CR47","first-page":"754","volume":"18","author":"RM Irving","year":"1997","unstructured":"Irving RM, Harada T, Moffat DA, Hardy DG, Whittaker JL, Xuereb JH, Maher ER (1997) Somatic neurofibromatosis type 2 gene mutations and growth characteristics in vestibular schwannoma. Am J Otolaryngol 18:754\u2013760","journal-title":"Am J Otolaryngol"},{"key":"4728_CR48","first-page":"60","volume":"30","author":"T Zhao","year":"2008","unstructured":"Zhao T, Xia WH, Zheng MQ, Lu CQ, Han X, Sun YJ (2008) Surgical excision promotes tumor growth and metastasis by promoting expression of MMP-9 and VEGF in a breast cancer model. Exp Oncol 30:60\u201364","journal-title":"Exp Oncol"},{"key":"4728_CR49","doi-asserted-by":"crossref","first-page":"255","DOI":"10.3171\/jns.2007.106.2.255","volume":"106","author":"WH Slattery 3rd","year":"2007","unstructured":"Slattery WH 3rd, 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\u2013260","journal-title":"J Neurosurg"},{"key":"4728_CR50","doi-asserted-by":"crossref","first-page":"466","DOI":"10.1097\/MAO.0b013e318248eaaa","volume":"33","author":"JR Tysome","year":"2012","unstructured":"Tysome JR, Macfarlane R, Durie-Gair J, Donnelly N, Mannion R, Knight R, Harris F, Vanat ZH, Tam YC, Burton K, Hensiek A, Raymond FL, Moffat DA, Axon PR (2012) Surgical management of vestibular schwannomas and hearing rehabilitation in neurofibromatosis type 2. Otol Neurotol 33:466\u2013472","journal-title":"Otol Neurotol"},{"key":"4728_CR51","doi-asserted-by":"crossref","first-page":"237","DOI":"10.1080\/00016489.1992.11665411","volume":"112","author":"ME Wigand","year":"1992","unstructured":"Wigand ME, Haid T, Goertzen W, Wolf S (1992) Preservation of hearing in bilateral acoustic neurinomas by deliberate partial resection. Acta Otolaryngol 112:237\u2013241","journal-title":"Acta Otolaryngol"},{"key":"4728_CR52","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1007\/s11060-019-03234-8","volume":"144","author":"VM Lu","year":"2019","unstructured":"Lu VM, Ravindran K, Graffeo CS, Perry A, Van Gompel JJ, Daniels DJ, Link MJ (2019) Efficacy and safety of bevacizumab for vestibular schwannoma in neurofibromatosis type 2: a systematic review and meta-analysis of treatment outcomes. J Neuro-Oncol 144:239\u2013248","journal-title":"J Neuro-Oncol"},{"key":"4728_CR53","doi-asserted-by":"crossref","unstructured":"Emmanouil B, Houston R, May A, Ramsden JD, Hanemann CO, Halliday D, Parry A, Mackeith S (2018) Progression of hearing loss in neurofibromatosis type 2 according to genetic severity. Laryngoscope","DOI":"10.1002\/lary.27586"},{"key":"4728_CR54","doi-asserted-by":"crossref","first-page":"657","DOI":"10.1136\/jmedgenet-2017-104519","volume":"54","author":"D Halliday","year":"2017","unstructured":"Halliday D, Emmanouil B, Pretorius P, MacKeith S, Painter S, Tomkins H, Evans DG, Parry A (2017) Genetic Severity Score predicts clinical phenotype in NF2. J Med Genet 54:657\u2013664","journal-title":"J Med Genet"},{"key":"4728_CR55","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1097\/00129492-200501000-00017","volume":"26","author":"P Caye-Thomasen","year":"2005","unstructured":"Caye-Thomasen P, Werther K, Nalla A, Bog-Hansen TC, Nielsen HJ, Stangerup SE, Thomsen J (2005) VEGF and VEGF receptor-1 concentration in vestibular schwannoma homogenates correlates to tumor growth rate. Otol Neurotol 26:98\u2013101","journal-title":"Otol Neurotol"},{"key":"4728_CR56","doi-asserted-by":"crossref","first-page":"3483","DOI":"10.1158\/0008-5472.CAN-09-3107","volume":"70","author":"HK Wong","year":"2010","unstructured":"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\u20133493","journal-title":"Cancer Res"}],"container-title":["Child's Nervous System"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00381-020-04728-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00381-020-04728-x\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00381-020-04728-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,6,15]],"date-time":"2021-06-15T23:45:32Z","timestamp":1623800732000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00381-020-04728-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,6,16]]},"references-count":56,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2020,10]]}},"alternative-id":["4728"],"URL":"https:\/\/doi.org\/10.1007\/s00381-020-04728-x","relation":{},"ISSN":["0256-7040","1433-0350"],"issn-type":[{"value":"0256-7040","type":"print"},{"value":"1433-0350","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,6,16]]},"assertion":[{"value":"10 May 2020","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"4 June 2020","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"16 June 2020","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"These retrospective studies were approved by the ethics board of the Medical Faculty and University Hospital of T\u00fcbingen.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Compliance with ethical standards"}},{"value":"The authors declare that they have no conflict of interest.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}]}}