Management of neurofibromatosis type 1-associated plexiform neurofibromas.
MEK inhibitor
neurofibroma
neurofibromatosis 1
plexiform
review
Journal
Neuro-oncology
ISSN: 1523-5866
Titre abrégé: Neuro Oncol
Pays: England
ID NLM: 100887420
Informations de publication
Date de publication:
02 11 2022
02 11 2022
Historique:
pubmed:
4
6
2022
medline:
4
11
2022
entrez:
3
6
2022
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 35657359
pii: 6601427
doi: 10.1093/neuonc/noac146
pmc: PMC9629437
doi:
Substances chimiques
Protein Kinase Inhibitors
0
Types de publication
Review
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1827-1844Subventions
Organisme : NICHD NIH HHS
ID : P50 HD105351
Pays : United States
Informations de copyright
Published by Oxford University Press on behalf of the Society for Neuro-Oncology 2022.
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