{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,31]],"date-time":"2026-10-31T01:45:13Z","timestamp":1793411113165,"version":"4.5.0"},"reference-count":115,"publisher":"Oxford University Press (OUP)","issue":"11","funder":[{"DOI":"10.13039\/100004325","name":"AstraZeneca","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100004325","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100007174","name":"Array BioPharma","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007174","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100010544","name":"Exelixis","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100010544","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2022,11,2]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Plexiform Neurofibromas (PN) are a common manifestation of the genetic disorder neurofibromatosis type 1 (NF1). These benign nerve sheath tumors often cause significant morbidity, with treatment options limited historically to surgery. There have been tremendous advances over the past two decades in our understanding of PN, and the recent regulatory approvals of the MEK inhibitor selumetinib are reshaping the landscape for PN management. At present, there is no agreed upon PN definition, diagnostic evaluation, surveillance strategy, or clear indications for when to initiate treatment and selection of treatment modality. In this review, we address these questions via consensus recommendations from a panel of multidisciplinary NF1 experts.<\/jats:p>","DOI":"10.1093\/neuonc\/noac146","type":"journal-article","created":{"date-parts":[[2022,6,3]],"date-time":"2022-06-03T10:46:00Z","timestamp":1654253160000},"page":"1827-1844","source":"Crossref","is-referenced-by-count":178,"title":["Management of neurofibromatosis type 1-associated plexiform neurofibromas"],"prefix":"10.1093","volume":"24","author":[{"given":"Michael J","family":"Fisher","sequence":"first","affiliation":[{"name":"Division of Oncology, The Children\u2019s Hospital of Philadelphia and the University of Pennsylvania Perelman School of Medicine , Philadelphia, Pennsylvania , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1049-0993","authenticated-orcid":false,"given":"Jaishri O","family":"Blakeley","sequence":"additional","affiliation":[{"name":"Division of Neuro-Oncology, Department of Neurology, Johns Hopkins University School of Medicine , Baltimore, Maryland , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brian D","family":"Weiss","sequence":"additional","affiliation":[{"name":"Division of Oncology, Cincinnati Children\u2019s Hospital Medical Center, University of Cincinnati College of Medicine , Cincinnati, Ohio , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5323-6995","authenticated-orcid":false,"given":"Eva","family":"Dombi","sequence":"additional","affiliation":[{"name":"Pediatric Oncology Branch, National Cancer Institute , Bethesda, Maryland , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shivani","family":"Ahlawat","sequence":"additional","affiliation":[{"name":"Russell H. Morgan Department of Radiology & Radiological Science, Johns Hopkins University School of Medicine , Baltimore, Maryland , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Srivandana","family":"Akshintala","sequence":"additional","affiliation":[{"name":"Pediatric Oncology Branch, National Cancer Institute , Bethesda, Maryland , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Allan J","family":"Belzberg","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Johns Hopkins University School of Medicine , Baltimore, Maryland , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7595-7726","authenticated-orcid":false,"given":"Miriam","family":"Bornhorst","sequence":"additional","affiliation":[{"name":"Family Neurofibromatosis Institute, Center for Neuroscience and Behavioral Medicine,Children\u2019s National Hospital, Washington, District of Columbia , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miriam A","family":"Bredella","sequence":"additional","affiliation":[{"name":"Department of Radiology, Massachusetts General Hospital and Harvard Medical School , Boston, Massachusetts , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wenli","family":"Cai","sequence":"additional","affiliation":[{"name":"Department of Radiology, Massachusetts General Hospital and Harvard Medical School , Boston, Massachusetts , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rosalie E","family":"Ferner","sequence":"additional","affiliation":[{"name":"Neurofibromatosis Service, Department of Neurology, Guy\u2019s Hospital, Guy\u2019s & St. Thomas\u2019 NHS Foundation Trust , London , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea M","family":"Gross","sequence":"additional","affiliation":[{"name":"Pediatric Oncology Branch, National Cancer Institute , Bethesda, Maryland , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gordon J","family":"Harris","sequence":"additional","affiliation":[{"name":"Department of Radiology, Massachusetts General Hospital and Harvard Medical School , Boston, Massachusetts , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Listernick","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Ann & Robert H. Lurie Children\u2019s Hospital of Chicago, Feinberg School of Medicine, Northwestern University , Chicago, Illinois , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ina","family":"Ly","sequence":"additional","affiliation":[{"name":"Stephen E. and Catherine Pappas Center for Neuro-Oncology, Massachusetts General Hospital , Boston, Massachusetts , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Staci","family":"Martin","sequence":"additional","affiliation":[{"name":"Pediatric Oncology Branch, National Cancer Institute , Bethesda, Maryland , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Victor F","family":"Mautner","sequence":"additional","affiliation":[{"name":"Department of Neurology, University Medical Center Hamburg-Eppendorf , Hamburg , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johannes M","family":"Salamon","sequence":"additional","affiliation":[{"name":"Department for Diagnostic and Interventional Radiology and Nuclear Medicine, University Medical Center Hamburg-Eppendorf , Hamburg , Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kilian E","family":"Salerno","sequence":"additional","affiliation":[{"name":"Radiation Oncology Branch, National Cancer Institute , Bethesda, Maryland , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert J","family":"Spinner","sequence":"additional","affiliation":[{"name":"Department of Neurologic Surgery, Mayo Clinic , Rochester, Minnesota , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Verena","family":"Staedtke","sequence":"additional","affiliation":[{"name":"Division of Neuro-Oncology, Department of Neurology, Johns Hopkins University School of Medicine , Baltimore, Maryland , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole J","family":"Ullrich","sequence":"additional","affiliation":[{"name":"Department of Neurology, Boston Children\u2019s Hospital , Boston, Massachusetts , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Meena","family":"Upadhyaya","sequence":"additional","affiliation":[{"name":"Division of Cancer and Genetics, Cardiff University , Wales , UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pamela L","family":"Wolters","sequence":"additional","affiliation":[{"name":"Pediatric Oncology Branch, National Cancer Institute , Bethesda, Maryland , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaleb","family":"Yohay","sequence":"additional","affiliation":[{"name":"Grossman School of Medicine, Department of Neurology , New York, New York , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brigitte C","family":"Widemann","sequence":"additional","affiliation":[{"name":"Pediatric Oncology Branch, National Cancer Institute , Bethesda, Maryland , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2022,6,3]]},"reference":[{"key":"2022110209031739200_CIT0001","doi-asserted-by":"crossref","first-page":"469","DOI":"10.1146\/annurev-pathol-011811-132441","article-title":"Pathogenesis of plexiform neurofibroma: tumor-stromal\/hematopoietic interactions in tumor progression","volume":"7","author":"Staser","year":"2012","journal-title":"Annu Rev Pathol."},{"issue":"1","key":"2022110209031739200_CIT0002","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1016\/S0165-4608(99)00006-0","article-title":"Allelic loss of the NF1 gene in NF1-associated plexiform neurofibromas","volume":"113","author":"Kluwe","year":"1999","journal-title":"Cancer Genet Cytogenet."},{"issue":"20","key":"2022110209031739200_CIT0003","doi-asserted-by":"crossref","first-page":"3055","DOI":"10.1093\/hmg\/9.20.3055","article-title":"Schwann cells harbor the somatic NF1 mutation in neurofibromas: evidence of two different Schwann cell subpopulations","volume":"9","author":"Serra","year":"2000","journal-title":"Hum Mol Genet."},{"issue":"3","key":"2022110209031739200_CIT0004","doi-asserted-by":"crossref","first-page":"437","DOI":"10.1016\/j.cell.2008.08.041","article-title":"Nf1-dependent tumors require a microenvironment containing Nf1+\/-- and c-kit-dependent bone marrow","volume":"135","author":"Yang","year":"2008","journal-title":"Cell."},{"issue":"Pt 6","key":"2022110209031739200_CIT0005","doi-asserted-by":"crossref","first-page":"1355","DOI":"10.1093\/brain\/111.6.1355","article-title":"Von Recklinghausen neurofibromatosis. 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