Medulloblastoma of early childhood. Salvage and outcome after initial CSI sparing approaches
Affiliation: Alberta Children’s Hospital, Calgary, Canada
All publications: Lucie Lafay-Cousin
All publications: Daynielle-Rose Cansanay
All publications: Craig Erker
All publications: Erin Ratterman
All publications: Mary Pat Schlosser
All publications: Liana Nobre
All publications: Rebecca Ronsley
All publications: Vanan Magimairajan
All publications: Natasha Pillay-Smiley
All publications: Andrew Cluster
All publications: Susan Chi
All publications: Annalise Bracher
All publications: Lindsey Hoffman
All publications: Akanksha Senapati
All publications: Marie Simbozel
All publications: Martin Minarek
All publications: Andrea M Cappellano
All publications: Kenneth J Cohen
All publications: Vicente Santa-Maria Lopez
All publications: Andres Morales-La Madrid
All publications: Chantel Cacciotti
All publications: Lorena Baroni
All publications: Ralph Salloum
All publications: Ashley S Margol
All publications: George Michaiel
All publications: Sylvia Cheng
All publications: Juliette Hukin
All publications: Dolly Aguilera
All publications: Claire Mazewski
All publications: Cassie Kline
All publications: Jonathan L. Finlay
All publications: Mohamed S. Abdelbaki
All publications: Jeffrey C. Murray
All publications: Kathleen Dorris
All publications: Bruce Crooks
All publications: Kevin F. Ginn
All publications: Nisreen Amayiri
All publications: Alvaro Lassaletta
All publications: Jordan R. Hansford
All publications: Sara Khan
All publications: Stephen W. Gilheeney
All publications: Michal Zapotocky
All publications: Valérie Larouche
All publications: Shafqat Shah
All publications: Amar Gajjar
All publications: Sébastien Perreault
All publications: Sabine Mueller
All publications: Zhihong J. Wang
All publications: Kara Matheson
All publications: Simon Bailey
All publications: Steven C. Clifford
All publications: Giles Robinson
All publications: Carlo Dufour
All publications: Stefan Rutkowski
All publications: Eric Bouffet
Abstract
Introduction: Infant medulloblastoma (iMB) is usually treated without craniospinal irradiation (CSI) to avoid neurocognitive late effects. Unfortunately, many of them relapse. In a previous cohort of 380 relapse iMB, initially treated without CSI, we reported a 3-year post relapse survival (PRS) of 52.4%. We described differences in PRS and salvage therapies according to molecular subgrouping.
Methodology: In this extended retrospective cohort, we explored salvage management of SHH/Nodular Desmoplastic (ND) iMB after initial CSI avoidance and for group 4 iMB, we investigated outcome after high dose chemotherapy (HDC) and described salvage management.
Results: For 147 SHH/ND iMB, median time to relapse was 14.7 months (range: 1-139.7). Relapse was local in 40.8%. Of 129 who received curative salvage therapy, half underwent surgery and 74.4% received radiotherapy (CSI 81.3%; median dose: 36 Gy). Patients with localized relapse were more likely to receive CSI dose ≤24Gy or focal RT (p<0.001; p<0.008). The 3-year PRS was 61.6% For the 36 group 4 iMB, 19(52.8%) relapsed (median time: 21 months) and 5-years PFS was 42.2%.
Relapse was local in 42.1%. Patients treated with 3 cycles of HDC had a better PFS compared to 1 or 2 cycles (p=0.001). 17/19 patients received salvage RT (median CSI dose of 36Gy). However, 7 received CSI dose < 24Gy and 2 underwent focal RT. Three years PRS was 70.2%. No prognostic factor was associated with PRS. The 5 yrs OS for the entire cohort was 71.6%.
Conclusion: Survival of relapsed SHH iMB is not satisfactory and relies on neurotoxic CSI. Maximizing upfront cure to avoid relapse is crucial. CSI dose ≤ 24 G can be considered for localized relapse. For Group4 iMB, HDC alone was associated with a high relapse rate but 70.5% were salvaged with RT. Such a strategy may be discussed for the youngest of group 4 iMB.