Pattern of Relapse and Treatment Response in WNT-Activated Medulloblastoma.

Journal Article (Journal Article)

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.

Full Text

Duke Authors

Cited Authors

  • Nobre, L; Zapotocky, M; Khan, S; Fukuoka, K; Fonseca, A; McKeown, T; Sumerauer, D; Vicha, A; Grajkowska, WA; Trubicka, J; Li, KKW; Ng, H-K; Massimi, L; Lee, JY; Kim, S-K; Zelcer, S; Vasiljevic, A; Faure-Conter, C; Hauser, P; Lach, B; van Veelen-Vincent, M-L; French, PJ; Van Meir, EG; Weiss, WA; Gupta, N; Pollack, IF; Hamilton, RL; Nageswara Rao, AA; Giannini, C; Rubin, JB; Moore, AS; Chambless, LB; Vibhakar, R; Ra, YS; Massimino, M; McLendon, RE; Wheeler, H; Zollo, M; Ferruci, V; Kumabe, T; Faria, CC; Sterba, J; Jung, S; López-Aguilar, E; Mora, J; Carlotti, CG; Olson, JM; Leary, S; Cain, J; Krskova, L; Zamecnik, J; Hawkins, CE; Tabori, U; Huang, A; Bartels, U; Northcott, PA; Taylor, MD; Yip, S; Hansford, JR; Bouffet, E; Ramaswamy, V

Published Date

  • June 23, 2020

Published In

Volume / Issue

  • 1 / 3

PubMed ID

  • 32743560

Pubmed Central ID

  • PMC7394286

Electronic International Standard Serial Number (EISSN)

  • 2666-3791

Digital Object Identifier (DOI)

  • 10.1016/j.xcrm.2020.100038


  • eng

Conference Location

  • United States