Management of neurofibromatosis type 1-associated plexiform neurofibromas

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...

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Published in:Neuro-oncology (Charlottesville, Va.) Va.), 2022-11, Vol.24 (11), p.1827-1844
Main Authors: Fisher, Michael J, Blakeley, Jaishri O, Weiss, Brian D, Dombi, Eva, Ahlawat, Shivani, Akshintala, Srivandana, Belzberg, Allan J, Bornhorst, Miriam, Bredella, Miriam A, Cai, Wenli, Ferner, Rosalie E, Gross, Andrea M, Harris, Gordon J, Listernick, Robert, Ly, Ina, Martin, Staci, Mautner, Victor F, Salamon, Johannes M, Salerno, Kilian E, Spinner, Robert J, Staedtke, Verena, Ullrich, Nicole J, Upadhyaya, Meena, Wolters, Pamela L, Yohay, Kaleb, Widemann, Brigitte C
Format: Article
Language:English
Subjects:
Humans
Nerve Sheath Neoplasms
Neurofibroma, Plexiform - pathology
Neurofibromatosis 1 - pathology
Protein Kinase Inhibitors
Reviews
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Summary: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.
ISSN:1522-8517
1523-5866
DOI:10.1093/neuonc/noac146