| Autor: |
Cynthia Hawkins, Marilyn M. Li, Ana Guerreiro Stucklin, Matthias A. Karajannis, Annie Huang, Christopher Li, Lili-Naz Hazrati, Sanja Pajovic, Abhaya V. Kulkarni, Javal Sheth, James T. Rutka, Ibrahim Qaddoumi, Angela J. Waanders, Michael D. Taylor, Derek S. Tsang, Ruth G. Tatevossian, Robert Siddaway, James M. Drake, Uri Tabori, Anthony Arnoldo, Mary Shago, Ute Bartels, Colleen D’Arcy, David W. Ellison, Vijay Ramaswamy, Stephen Gilheeney, Manohar Shroff, Scott Ryall, Peter B. Dirks, Michal Zapotocky, Eric Bouffet, Lea F. Surrey, Alvaro Lassaletta, Tejus Bale, Liana Nobre, Monique Johnson, Marc K. Rosenblum, Julie Bennett, George M. Ibrahim, Mariarita Santi, Normand Laperriere, Suzanne Laughlin, Kohei Fukuoka, Wilda Orisme, Paul E. Kowalski |
| Rok vydání: |
2020 |
| Předmět: |
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| Zdroj: |
Cancer Cell |
| ISSN: |
1878-3686 |
| Popis: |
Pediatric low-grade gliomas (pLGG) are frequently driven by genetic alterations in the RAS-mitogen-activated protein kinase (RAS/MAPK) pathway yet show unexplained variability in their clinical outcome. To address this, we characterized a cohort of >1,000 clinically annotated pLGG. Eighty-four percent of cases harbored a driver alteration, while those without an identified alteration also often exhibited upregulation of the RAS/MAPK pathway. pLGG could be broadly classified based on their alteration type. Rearrangement-driven tumors were diagnosed at a younger age, enriched for WHO grade I histology, infrequently progressed, and rarely resulted in death as compared with SNV-driven tumors. Further sub-classification of clinical-molecular correlates stratified pLGG into risk categories. These data highlight the biological and clinical differences between pLGG subtypes and opens avenues for future treatment refinement. |
| Databáze: |
OpenAIRE |
| Externí odkaz: |
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