Management of neurofibromatosis type 1-associated plexiform neurofibromas

Michael J. Fisher; Jaishri O. Blakeley; Brian D. Weiss; Eva Dombi; Shivani Ahlawat; Srivandana Akshintala; Allan J. Belzberg; Miriam Bornhorst; Miriam A. Bredella; Wenli Cai; Rosalie E. Ferner; Andrea M. Gross; Gordon J. Harris; Robert Listernick; Ina Ly; Staci Martin; Victor F. Mautner; Johannes M. Salamon; Kilian E. Salerno; Robert J. Spinner; Verena Staedtke; Nicole J. Ullrich; Meena Upadhyaya; Pamela L. Wolters; Kaleb Yohay; Brigitte C. Widemann

doi:10.1093/neuonc/noac146

Management of neurofibromatosis type 1-associated plexiform neurofibromas

Michael J. Fisher, Jaishri O. Blakeley, Brian D. Weiss, Eva Dombi, Shivani Ahlawat, Srivandana Akshintala, Allan J. Belzberg, Miriam Bornhorst, Miriam A. Bredella, Wenli Cai, Rosalie E. Ferner, Andrea M. Gross, Gordon J. Harris, Robert Listernick, Ina Ly, Staci Martin, Victor F. Mautner, Johannes M. Salamon, Kilian E. Salerno, Robert J. SpinnerVerena Staedtke, Nicole J. Ullrich, Meena Upadhyaya, Pamela L. Wolters, Kaleb Yohay, Brigitte C. Widemann

School of Medicine

Research output: Contribution to journal › Review article › peer-review

Abstract

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.

Original language	English (US)
Pages (from-to)	1827-1844
Number of pages	18
Journal	Neuro-oncology
Volume	24
Issue number	11
DOIs	https://doi.org/10.1093/neuonc/noac146
State	Published - Nov 1 2022

Keywords

MEK inhibitor
neurofibroma
neurofibromatosis 1
plexiform
review

ASJC Scopus subject areas

Clinical Neurology
Oncology
Cancer Research

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10.1093/neuonc/noac146

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Fisher, M. J., Blakeley, J. O., Weiss, B. D., Dombi, E., Ahlawat, S., Akshintala, S., Belzberg, A. J., Bornhorst, M., Bredella, M. A., Cai, W., Ferner, R. E., Gross, A. M., Harris, G. J., Listernick, R., Ly, I., Martin, S., Mautner, V. F., Salamon, J. M., Salerno, K. E., ... Widemann, B. C. (2022). Management of neurofibromatosis type 1-associated plexiform neurofibromas. Neuro-oncology, 24(11), 1827-1844. https://doi.org/10.1093/neuonc/noac146

Fisher, MJ, Blakeley, JO, Weiss, BD, Dombi, E, Ahlawat, S, Akshintala, S, Belzberg, AJ, Bornhorst, M, Bredella, MA, Cai, W, Ferner, RE, Gross, AM, Harris, GJ, Listernick, R, Ly, I, Martin, S, Mautner, VF, Salamon, JM, Salerno, KE, Spinner, RJ, Staedtke, V, Ullrich, NJ, Upadhyaya, M, Wolters, PL, Yohay, K & Widemann, BC 2022, 'Management of neurofibromatosis type 1-associated plexiform neurofibromas', Neuro-oncology, vol. 24, no. 11, pp. 1827-1844. https://doi.org/10.1093/neuonc/noac146

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title = "Management of neurofibromatosis type 1-associated plexiform neurofibromas",

abstract = "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.",

keywords = "MEK inhibitor, neurofibroma, neurofibromatosis 1, plexiform, review",

author = "Fisher, {Michael J.} and Blakeley, {Jaishri O.} and Weiss, {Brian D.} and Eva Dombi and Shivani Ahlawat and Srivandana Akshintala and Belzberg, {Allan J.} and Miriam Bornhorst and Bredella, {Miriam A.} and Wenli Cai and Ferner, {Rosalie E.} and Gross, {Andrea M.} and Harris, {Gordon J.} and Robert Listernick and Ina Ly and Staci Martin and Mautner, {Victor F.} and Salamon, {Johannes M.} and Salerno, {Kilian E.} and Spinner, {Robert J.} and Verena Staedtke and Ullrich, {Nicole J.} and Meena Upadhyaya and Wolters, {Pamela L.} and Kaleb Yohay and Widemann, {Brigitte C.}",

note = "Publisher Copyright: {\textcopyright} 2022 Published by Oxford University Press on behalf of the Society for Neuro-Oncology.",

year = "2022",

month = nov,

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doi = "10.1093/neuonc/noac146",

language = "English (US)",

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AU - Blakeley, Jaishri O.

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AU - Ahlawat, Shivani

AU - Akshintala, Srivandana

AU - Belzberg, Allan J.

AU - Bornhorst, Miriam

AU - Bredella, Miriam A.

AU - Cai, Wenli

AU - Ferner, Rosalie E.

AU - Gross, Andrea M.

AU - Harris, Gordon J.

AU - Listernick, Robert

AU - Ly, Ina

AU - Martin, Staci

AU - Mautner, Victor F.

AU - Salamon, Johannes M.

AU - Salerno, Kilian E.

AU - Spinner, Robert J.

AU - Staedtke, Verena

AU - Ullrich, Nicole J.

AU - Upadhyaya, Meena

AU - Wolters, Pamela L.

AU - Yohay, Kaleb

AU - Widemann, Brigitte C.

PY - 2022/11/1

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AB - 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.

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Management of neurofibromatosis type 1-associated plexiform neurofibromas

Abstract

Keywords

ASJC Scopus subject areas

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