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  • Fisher, Michael J.The Children's Hospital of Philadelphia (author)

Integrated molecular and clinical analysis of low-grade gliomas in children with neurofibromatosis type 1 (NF1)

  • Article/chapterEnglish2021

Publisher, publication year, extent ...

  • 2021-02-14
  • Springer Science and Business Media LLC,2021

Numbers

  • LIBRIS-ID:oai:lup.lub.lu.se:6e2eb44b-02cc-4a93-9698-022f66f8b961
  • https://lup.lub.lu.se/record/6e2eb44b-02cc-4a93-9698-022f66f8b961URI
  • https://doi.org/10.1007/s00401-021-02276-5DOI

Supplementary language notes

  • Language:English
  • Summary in:English

Part of subdatabase

Classification

  • Subject category:art swepub-publicationtype
  • Subject category:ref swepub-contenttype

Notes

  • Low-grade gliomas (LGGs) are the most common childhood brain tumor in the general population and in individuals with the Neurofibromatosis type 1 (NF1) cancer predisposition syndrome. Surgical biopsy is rarely performed prior to treatment in the setting of NF1, resulting in a paucity of tumor genomic information. To define the molecular landscape of NF1-associated LGGs (NF1-LGG), we integrated clinical data, histological diagnoses, and multi-level genetic/genomic analyses on 70 individuals from 25 centers worldwide. Whereas, most tumors harbored bi-allelic NF1 inactivation as the only genetic abnormality, 11% had additional mutations. Moreover, tumors classified as non-pilocytic astrocytoma based on DNA methylation analysis were significantly more likely to harbor these additional mutations. The most common secondary alteration was FGFR1 mutation, which conferred an additional growth advantage in multiple complementary experimental murine Nf1 models. Taken together, this comprehensive characterization has important implications for the management of children with NF1-LGG, distinct from their sporadic counterparts.

Subject headings and genre

Added entries (persons, corporate bodies, meetings, titles ...)

  • Jones, David T.W.Hopp Children’s Cancer Center Heidelberg (KiTZ),German Cancer Research Centre (author)
  • Li, YimeiUniversity of Pennsylvania (author)
  • Guo, XiaofanWashington University in St. Louis (author)
  • Sonawane, Poonam S.The Children's Hospital of Philadelphia (author)
  • Waanders, Angela J.Ann and Robert H. Lurie Children's Hospital,The Children's Hospital of Philadelphia (author)
  • Phillips, Joanna J.University of California, San Francisco (author)
  • Weiss, William A.University of California, San Francisco (author)
  • Resnick, Adam C.The Children's Hospital of Philadelphia (author)
  • Gosline, SaraSage Bionetworks,Pacific Northwest National Laboratory (author)
  • Banerjee, JinetaSage Bionetworks (author)
  • Guinney, JustinSage Bionetworks (author)
  • Gnekow, AstridUniversity of Augsburg (author)
  • Kandels, DanielaUniversity of Augsburg (author)
  • Foreman, Nicholas K.University of Colorado (author)
  • Korshunov, AndreyHeidelberg University (author)
  • Ryzhova, MarinaNN Burdenko Neurosurgical Research Centre (author)
  • Massimi, LucaPoliclinico Universitario Agostino Gemelli (author)
  • Gururangan, SriUF Health Shands Cancer Hospital (author)
  • Kieran, Mark W.Bristol-Myers Squibb,Dana-Farber Cancer Institute (author)
  • Wang, ZhihongChildren’s Hospital of Richmond,Children’s Hospital of Michigan (author)
  • Fouladi, MaryamCincinnati Children's Hospital Medical Center (author)
  • Sato, MarikoUniversity of Iowa Stead Family Children’s Hospital (author)
  • Øra, IngridLund University,Lunds universitet,Childhood Cancer Research Unit,Forskargrupper vid Lunds universitet,LUCC: Lunds universitets cancercentrum,Övriga starka forskningsmiljöer,Lund University Research Groups,LUCC: Lund University Cancer Centre,Other Strong Research Environments(Swepub:lu)molm-ior (author)
  • Holm, StefanKarolinska University Hospital (author)
  • Markham, Stephen J.The Children's Hospital of Philadelphia (author)
  • Beck, PengboHopp Children’s Cancer Center Heidelberg (KiTZ),German Cancer Research Centre (author)
  • Jäger, NatalieHopp Children’s Cancer Center Heidelberg (KiTZ),German Cancer Research Centre (author)
  • Wittmann, AndreaHopp Children’s Cancer Center Heidelberg (KiTZ) (author)
  • Sommerkamp, Alexander C.Hopp Children’s Cancer Center Heidelberg (KiTZ),German Cancer Research Centre (author)
  • Sahm, FelixHeidelberg University (author)
  • Pfister, Stefan M.Hopp Children’s Cancer Center Heidelberg (KiTZ),German Cancer Research Centre,University Hospital Heidelberg (author)
  • Gutmann, David H.Washington University in St. Louis (author)
  • The Children's Hospital of PhiladelphiaHopp Children’s Cancer Center Heidelberg (KiTZ) (creator_code:org_t)

Related titles

  • In:Acta Neuropathologica: Springer Science and Business Media LLC141:4, s. 605-6170001-63221432-0533

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