Multicentre evaluation of the management of children with high risk medulloblastoma: Real world performance of the SJMB03 and COG-99701 protocols

Several treatment protocols are used for the treatment of high-risk medulloblastoma (HR-MB). In 2015, the UK Children’s Cancer and Leukaemia Group issued guidance recommending treatment as per the SJMB03 protocol, whilst also recognising that the COG-99701 protocol may be used. Patients were defined...

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Main Authors: John R. Apps, Jack Goddard, Shaimaa Sahmoud, Katherine Green, Kriti Hedge, Claire Keeling, Anoop Cherungonath, Banuja Srikumar, Blanche Lumb, Jennifer Whitby, James Hayden, Jenny Gains, Henry Mandeville, Lewis Joanne, Barry Pizer, John Paul Kilday, Steven C. Clifford, Simon Bailey, Mette Jorgensen, Fernando Carceller, Jenny Adamski
Format: Article
Language:English
Published: Elsevier 2025-12-01
Series:EJC Paediatric Oncology
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Online Access:http://www.sciencedirect.com/science/article/pii/S2772610X2500100X
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Summary:Several treatment protocols are used for the treatment of high-risk medulloblastoma (HR-MB). In 2015, the UK Children’s Cancer and Leukaemia Group issued guidance recommending treatment as per the SJMB03 protocol, whilst also recognising that the COG-99701 protocol may be used. Patients were defined as high-risk if metastatic at presentation, large-cell/anaplastic histology, MYC amplification, significant residual disease or MYCN amplification. Recently, the latter two only define high risk if other adverse features are present. Methods: Retrospective multi-centre service evaluation of treatment of HR-MB at five UK centres. Patients were included if treated as per SJMB03 or COG-99701. Patients were excluded if initially treated for standard-risk medulloblastoma and subsequently treated with these protocols due to upstaging or disease progression. Results: 58 patients were identified: 26 treated as per SJMB03, 32 as per COG-99701. 5-year OS was 83 % (95 %CI 73–94 %) and 5-year PFS was 65 % (53–80 %). For patients treated as per SJMB03, 5-year OS and PFS were 80 % (65–97 %) and 75 % (60–95 %) respectively; for patients treated as per COG-99701, 5-year OS and PFS were 85 % (73–100 %) and 60 % (43–83 %). There was no significant difference in outcomes between protocols. There was a higher incidence of grade 3/4 ototoxicity (44 % vs 6 %, p = 0.001) and admission to paediatric intensive care (19 % vs 0 %, p = 0.014) in patients treated as per SJMB03 compared to COG-99701. Conclusion: These real-world outcomes are consistent with the published literature on HR-MB patients treated with these protocols within clinical trials, and provide important evidence to inform their use in routine practice.
ISSN:2772-610X