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Pattern of Relapse and Treatment Response in WNT-Activated Medulloblastoma

2020; Elsevier BV; Volume: 1; Issue: 3 Linguagem: Inglês

10.1016/j.xcrm.2020.100038

2666-3791

Liana Nobre, Michal Zápotocký, Sara Khan, Kohei Fukuoka, Adriana Fonseca, Tara McKeown, David Sumerauer, Aleš Vícha, Wiesława Grajkowska, Joanna Trubicka, Kay Ka Wai Li, Ho‐Keung Ng, Luca Massimi, Ji Yeoun Lee, Seung-Ki Kim, Shayna Zelcer, Alexandre Vasiljevic, Cécile Faure‐Conter, Péter Hauser, Bolesław Lach, Marie‐Lise C. van Veelen, Pim J. French, Erwin G. Van Meir, William A. Weiss, Nalin Gupta, Ian F. Pollack, Ronald L. Hamilton, Amulya A. Nageswara Rao, Caterina Giannini, Joshua B. Rubin, Andrew S. Moore, Lola B. Chambless, Rajeev Vibhakar, Young Seob Shin, Maura Massimino, Roger E. McLendon, Helen Wheeler, Massimo Zollo, Veronica Ferruci, Toshihiro Kumabe, Cláudia C. Faria, Jaroslav Štěrba, Shin Jung, Enrique López‐Aguilar, Jaume Mora, Carlos Gilberto Carlotti, James M. Olson, Sarah Leary, Jason E. Cain, Lenka Krsková, Josef Zámečnı́k, Cynthia Hawkins, Uri Tabori, Annie Huang, Ute Bartels, Paul A. Northcott, Michael D. Taylor, Stephen Yip, Jordan R. Hansford, Éric Bouffet, Vijay Ramaswamy,

Brain Metastases and Treatment

Over the past decade, wingless-activated (WNT) medulloblastoma has been identified as a candidate for therapy de-escalation based on excellent survival; however, a paucity of relapses has precluded additional analyses of markers of relapse. To address this gap in knowledge, an international cohort of 93 molecularly confirmed WNT MB was assembled, where 5-year progression-free survival is 0.84 (95%, 0.763–0.925) with 15 relapsed individuals identified. Maintenance chemotherapy is identified as a strong predictor of relapse, with individuals receiving high doses of cyclophosphamide or ifosphamide having only one very late molecularly confirmed relapse (p = 0.032). The anatomical location of recurrence is metastatic in 12 of 15 relapses, with 8 of 12 metastatic relapses in the lateral ventricles. Maintenance chemotherapy, specifically cumulative cyclophosphamide doses, is a significant predictor of relapse across WNT MB. Future efforts to de-escalate therapy need to carefully consider not only the radiation dose but also the chemotherapy regimen and the propensity for metastatic relapses.