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The name of \u201cneurofibromatosis type 2\u201d has been changed to NF2-related schwannomatosis, to reflect the fact that neurofibromas do not occur in this syndrome and therefore the name \u201cNeurofibromatosis\u201d is factually incorrect. Furthermore, multiple schwannomas, a hallmark feature of NF2, can also occur in patients with mutations in genes including SMARCB1 and LZTR1, all exhibiting overlapping clinical features. Current understanding suggests that schwannomatosis (SWN) encompasses a range of clinical presentations consisting of clearly defined, separate subtypes which share a common phenotype of schwannomas. Recognizing these newly emerging subtypes, the International Consensus Group on Neurofibromatosis Diagnostic Criteria (I-NF-DC) proposed a revised nomenclature for NF2 and related disorders in 2022. This review article focuses on this critical update in diagnostic terminology, highlighting the key gene-related SWN subtypes relevant to neuroradiologists. By emphasizing molecular testing alongside clinical features, the revised system facilitates a more precise diagnosis, potentially paving the way for personalized treatment strategies. Additionally, the flexible structure accommodates future discoveries of genes associated with SWN.<\/jats:p>","DOI":"10.1177\/19714009251313510","type":"journal-article","created":{"date-parts":[[2025,1,9]],"date-time":"2025-01-09T10:21:02Z","timestamp":1736418062000},"page":"533-542","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":11,"title":["Classification of schwannomas and the new naming convention for \u201cneurofibromatosis-2\u201d: Genetic updates and international consensus recommendation"],"prefix":"10.1177","volume":"38","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-3796-8273","authenticated-orcid":false,"given":"Pranjal","family":"Rai","sequence":"first","affiliation":[{"name":"Department of Radiology, Tata Memorial Hospital, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3170-242X","authenticated-orcid":false,"given":"Girish","family":"Bathla","sequence":"additional","affiliation":[{"name":"Department of Radiology, Mayo Clinic, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neetu","family":"Soni","sequence":"additional","affiliation":[{"name":"Department of Radiology, Mayo Clinic, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amit","family":"Desai","sequence":"additional","affiliation":[{"name":"Department of Neuroradiology, Mayo Clinic, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dinesh","family":"Rao","sequence":"additional","affiliation":[{"name":"Department of Neuroradiology, Mayo Clinic, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Prasanna","family":"Vibhute","sequence":"additional","affiliation":[{"name":"Department of Neuroradiology, Mayo Clinic, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amit","family":"Agarwal","sequence":"additional","affiliation":[{"name":"Department of Neuroradiology, Mayo Clinic, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2025,1,9]]},"reference":[{"key":"e_1_3_3_2_2","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.37.12.897"},{"key":"e_1_3_3_3_2","doi-asserted-by":"publisher","DOI":"10.1038\/363515a0"},{"key":"e_1_3_3_4_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng.2855"},{"key":"e_1_3_3_5_2","doi-asserted-by":"publisher","DOI":"10.1086\/513207"},{"key":"e_1_3_3_6_2","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.40.6.459"},{"key":"e_1_3_3_7_2","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.2006.047753"},{"key":"e_1_3_3_8_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41436-019-0598-7"},{"key":"e_1_3_3_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.gim.2022.05.007"},{"key":"e_1_3_3_10_2","doi-asserted-by":"publisher","DOI":"10.1002\/humu.20679"},{"key":"e_1_3_3_11_2","doi-asserted-by":"publisher","DOI":"10.1038\/s41436-021-01170-5"},{"key":"e_1_3_3_12_2","doi-asserted-by":"publisher","DOI":"10.3171\/2015.11.JNS151766"},{"key":"e_1_3_3_13_2","doi-asserted-by":"publisher","DOI":"10.1111\/cge.13013"},{"key":"e_1_3_3_14_2","doi-asserted-by":"publisher","DOI":"10.1634\/theoncologist.2012-0162"},{"key":"e_1_3_3_15_2","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-4-16"},{"key":"e_1_3_3_16_2","doi-asserted-by":"publisher","DOI":"10.1186\/s12935-023-02940-8"},{"key":"e_1_3_3_17_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.wneu.2020.01.019"},{"key":"e_1_3_3_18_2","doi-asserted-by":"publisher","DOI":"10.1136\/jmedgenet-2015-103050"},{"key":"e_1_3_3_19_2","doi-asserted-by":"publisher","DOI":"10.1093\/neuonc\/nou317"},{"key":"e_1_3_3_20_2","doi-asserted-by":"publisher","DOI":"10.3171\/2014.3.PEDS13455"},{"key":"e_1_3_3_21_2","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2017.5464"},{"key":"e_1_3_3_22_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM198803173181106"},{"key":"e_1_3_3_23_2","doi-asserted-by":"publisher","DOI":"10.1136\/adc.81.6.496"},{"key":"e_1_3_3_24_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00381-020-04729-w"},{"key":"e_1_3_3_25_2","doi-asserted-by":"publisher","DOI":"10.1097\/00005072-199705000-00004"},{"key":"e_1_3_3_26_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1749-6632.1986.tb48058.x"},{"key":"e_1_3_3_27_2","doi-asserted-by":"publisher","DOI":"10.1136\/jmedgenet-2017-104519"},{"key":"e_1_3_3_28_2","volume-title":"GeneReviews\u00ae","author":"Dhamija R","year":"1993","unstructured":"Dhamija R, Plotkin S, Gomes A, et al. 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