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Update on pediatric cancer surveillance recommendations for patients with Neurofibromatosis type 1, Noonan syndrome, CBL syndrome, Costello syndrome, and related RASopathies

Neurofibromatosis type 1 (NF1), Noonan syndrome, and related syndromes, grouped as RASopathies, result from dysregulation of the RAS-MAPK pathway and demonstrate varied multisystemic clinical phenotypes. Together, RASopathies are among the more prevalent genetic cancer predisposition syndromes and r...

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Hauptverfasser: Perrino, Melissa (VerfasserIn) , Das, Anirban (VerfasserIn) , Scollon, Sarah R. (VerfasserIn) , Mitchell, Sarah G. (VerfasserIn) , Greer, Mary-Louise C. (VerfasserIn) , Yohe, Marielle E. (VerfasserIn) , Hansford, Jordan R. (VerfasserIn) , Kalish, Jennifer M. (VerfasserIn) , Schultz, Kris Ann P. (VerfasserIn) , MacFarland, Suzanne P. (VerfasserIn) , Kohlmann, Wendy K. (VerfasserIn) , Lupo, Philip J. (VerfasserIn) , Maxwell, Kara N. (VerfasserIn) , Pfister, Stefan (VerfasserIn) , Weksberg, Rosanna (VerfasserIn) , Michaeli, Orli (VerfasserIn) , Jongmans, Marjolijn C.J. (VerfasserIn) , Tomlinson, Gail E. (VerfasserIn) , Brzezinski, Jack (VerfasserIn) , Tabori, Uri (VerfasserIn) , Ney, Gina M. (VerfasserIn) , Gripp, Karen W. (VerfasserIn) , Gross, Andrea M. (VerfasserIn) , Widemann, Brigitte C. (VerfasserIn) , Stewart, Douglas R. (VerfasserIn) , Woodward, Emma R. (VerfasserIn) , Kratz, Christian P. (VerfasserIn)
Dokumenttyp: Article (Journal)
Sprache:Englisch
Veröffentlicht: 1 November 2024
In: Clinical cancer research
Year: 2024, Jahrgang: 30, Heft: 21, Pages: 4834-4843
ISSN:1557-3265
DOI:10.1158/1078-0432.CCR-24-1611
Online-Zugang:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1158/1078-0432.CCR-24-1611
Volltext
Verfasserangaben:Melissa R. Perrino, Anirban Das, Sarah R. Scollon, Sarah G. Mitchell, Mary-Louise C. Greer, Marielle E. Yohe, Jordan R. Hansford, Jennifer M. Kalish, Kris Ann P. Schultz, Suzanne P. MacFarland, Wendy K. Kohlmann, Philip J. Lupo, Kara N. Maxwell, Stefan M. Pfister, Rosanna Weksberg, Orli Michaeli, Marjolijn C.J. Jongmans, Gail E. Tomlinson, Jack Brzezinski, Uri Tabori, Gina M. Ney, Karen W. Gripp, Andrea M. Gross, Brigitte C. Widemann, Douglas R. Stewart, Emma R. Woodward, and Christian P. Kratz
Beschreibung
Zusammenfassung:Neurofibromatosis type 1 (NF1), Noonan syndrome, and related syndromes, grouped as RASopathies, result from dysregulation of the RAS-MAPK pathway and demonstrate varied multisystemic clinical phenotypes. Together, RASopathies are among the more prevalent genetic cancer predisposition syndromes and require nuanced clinical management. When compared with the general population, children with RASopathies are at significantly increased risk of benign and malignant neoplasms. In the past decade, clinical trials have shown that targeted therapies can improve outcomes for low-grade and benign neoplastic lesions but have their own challenges, highlighting the multidisciplinary care needed for such individuals, specifically those with NF1. This perspective, which originated from the 2023 American Association for Cancer Research Childhood Cancer Predisposition Workshop, serves to update pediatric oncologists, neurologists, geneticists, counselors, and other health care professionals on revised diagnostic criteria, review previously published surveillance guidelines, and harmonize updated surveillance recommendations for patients with NF1 or RASopathies.
Beschreibung:Gesehen am 11.06.2025
Beschreibung:Online Resource
ISSN:1557-3265
DOI:10.1158/1078-0432.CCR-24-1611

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