{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T14:36:31Z","timestamp":1787495791446,"version":"build-2736575974"},"reference-count":51,"publisher":"Korean Neurosurgical Society","issue":"3","license":[{"start":{"date-parts":[[2025,2,24]],"date-time":"2025-02-24T00:00:00Z","timestamp":1740355200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/3.0\/"}],"content-domain":{"domain":["jkns.or.kr"],"crossmark-restriction":true},"short-container-title":["J Korean Neurosurg Soc"],"published-print":{"date-parts":[[2025,5,1]]},"abstract":"<jats:p>Neurofibromatosis type 2 (NF2) is a rare genetic disorder caused by mutations in the &lt;i&gt;NF2&lt;\/i&gt; tumor suppressor gene, characterized by bilateral vestibular schwannomas and other central and peripheral nervous system tumors. Pediatric patients often present with more aggressive disease, greater tumor burdens, and increased morbidity compared to adults. Management requires a multidisciplinary approach that balances tumor control with functional preservation. While surgery and radiosurgery remain key treatment options, they carry risks such as hearing loss and malignant transformation of existing tumors. Bevacizumab and emerging therapies like gene therapy show promising therapeutic effects but are limited by variability in efficacy. Comprehensive care, including psychosocial support, is essential to improve clinical outcomes and quality of life for children with NF2.<\/jats:p>","DOI":"10.3340\/jkns.2024.0237","type":"journal-article","created":{"date-parts":[[2025,2,24]],"date-time":"2025-02-24T00:26:42Z","timestamp":1740356802000},"page":"278-285","update-policy":"https:\/\/doi.org\/10.3340\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Management Strategies of Neurofibromatosis Type 2 in Pediatric Patients : Challenges and Emerging Therapies"],"prefix":"10.3340","volume":"68","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1423-2681","authenticated-orcid":true,"given":"Jong Seok","family":"Lee","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1892","reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1177\/0883073816666736"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1016\/s0140-6736(09)60259-2"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1054\/bjoc.1999.1030"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.34359"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1001\/archopht.124.3.379"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1177\/01945998211012674"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuchi.2016.09.008"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1093\/noajnl\/vdad099"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.3171\/2014.3.peds13455"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.3171\/2012.3.jns111649"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.3390\/cancers16081479"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2005.036319"},{"key":"ref13","first-page":"vdad025","volume-title":"Radiation treatment of benign tumors in NF2-related-schwannomatosis: a national study of 266 irradiated patients showing a significant increase in malignancy\/malignant progression","author":"Evans","year":"2023"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.66.6.764"},{"key":"ref15","doi-asserted-by":"publisher","DOI":"10.1097\/00129492-200501000-00016"},{"key":"ref16","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2004.023705"},{"key":"ref17","doi-asserted-by":"publisher","DOI":"10.1158\/1078-0432.ccr-17-0590"},{"key":"ref18","doi-asserted-by":"publisher","DOI":"10.1038\/s41598-024-57401-7"},{"key":"ref19","doi-asserted-by":"publisher","DOI":"10.1097\/mao.0b013e3182355855"},{"key":"ref20","doi-asserted-by":"publisher","DOI":"10.1186\/s12935-023-02940-8"},{"key":"ref21","doi-asserted-by":"publisher","DOI":"10.1093\/neuonc\/noz153"},{"key":"ref22","doi-asserted-by":"publisher","DOI":"10.3171\/2019.6.peds1947"},{"key":"ref23","doi-asserted-by":"publisher","DOI":"10.1007\/s00381-020-04728-x"},{"key":"ref24","doi-asserted-by":"publisher","DOI":"10.1111\/cge.14310"},{"key":"ref25","doi-asserted-by":"publisher","DOI":"10.3171\/jns.1994.80.3.0541"},{"key":"ref26","doi-asserted-by":"publisher","DOI":"10.1227\/neu.0b013e31822f7d5f"},{"key":"ref27","doi-asserted-by":"publisher","DOI":"10.1093\/neuonc\/nos146"},{"key":"ref28","doi-asserted-by":"publisher","DOI":"10.1093\/nop\/npz076"},{"key":"ref29","doi-asserted-by":"publisher","DOI":"10.1080\/02688690902968844"},{"key":"ref30","first-page":"889","volume-title":"Neurofibromatosis type 2 vestibular schwannoma treatment: a review of the literature, trends, and outcomes","author":"Maniakas","year":"2014"},{"key":"ref31","first-page":"460","volume-title":"Stereotactic radiosurgery for vestibular schwannomas in patients with neurofibromatosis type 2: an analysis of tumor control, complications, and hearing preservation rates","author":"Mathieu","year":"2007"},{"key":"ref32","doi-asserted-by":"publisher","DOI":"10.1016\/j.wneu.2021.11.102"},{"key":"ref33","doi-asserted-by":"publisher","DOI":"10.1007\/s11060-016-2276-9"},{"key":"ref34","doi-asserted-by":"publisher","DOI":"10.1097\/mlg.0b013e31804b1ae7"},{"key":"ref35","doi-asserted-by":"publisher","DOI":"10.3171\/jns.2003.99.3.0480"},{"key":"ref36","doi-asserted-by":"publisher","DOI":"10.3171\/2020.4.jns201069"},{"key":"ref37","doi-asserted-by":"publisher","DOI":"10.1093\/jnen\/60.10.994"},{"key":"ref38","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.24036"},{"key":"ref39","doi-asserted-by":"publisher","DOI":"10.1093\/neuonc\/noad066"},{"key":"ref40","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000435746.02780.f6"},{"key":"ref41","first-page":"1967","volume-title":"Updated diagnostic criteria and nomenclature for neurofibromatosis type 2 and schwannomatosis: an international consensus recommendation","author":"Plotkin","year":"2022"},{"key":"ref42","doi-asserted-by":"publisher","DOI":"10.1002\/pon.5099"},{"key":"ref43","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.74.9.1288"},{"key":"ref44","doi-asserted-by":"publisher","DOI":"10.1055\/s-2005-837581"},{"key":"ref45","doi-asserted-by":"publisher","DOI":"10.3171\/ped.2007.106.4.255"},{"key":"ref46","doi-asserted-by":"publisher","DOI":"10.1200\/jco.2011.38.2614"},{"key":"ref47","doi-asserted-by":"publisher","DOI":"10.3171\/2009.10.jns091203"},{"key":"ref48","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-019-13640-1"},{"key":"ref49","doi-asserted-by":"publisher","DOI":"10.1007\/s11060-021-03910-8"},{"key":"ref50","doi-asserted-by":"publisher","DOI":"10.1097\/mao.0000000000003815"},{"key":"ref51","doi-asserted-by":"publisher","DOI":"10.1007\/s40487-024-00279-2"}],"container-title":["Journal of Korean Neurosurgical Society"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/jkns.or.kr\/upload\/pdf\/jkns-2024-0237.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/jkns.or.kr\/journal\/view.php?doi=10.3340\/jkns.2024.0237","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/jkns.or.kr\/upload\/pdf\/jkns-2024-0237.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,5,8]],"date-time":"2025-05-08T03:15:29Z","timestamp":1746674129000},"score":1,"resource":{"primary":{"URL":"http:\/\/jkns.or.kr\/journal\/view.php?doi=10.3340\/jkns.2024.0237"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,5,1]]},"references-count":51,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2025,5]]}},"URL":"https:\/\/doi.org\/10.3340\/jkns.2024.0237","relation":{},"ISSN":["2005-3711","1598-7876"],"issn-type":[{"value":"2005-3711","type":"print"},{"value":"1598-7876","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,5,1]]},"assertion":[{"value":"2024-12-30","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-02-20","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-02-24","order":2,"name":"published","label":"Published online","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-05-01","order":3,"name":"published","label":"Print publication","group":{"name":"publication_history","label":"Publication History"}}]}}