A clinical observational study of dinutuximab beta as first-line maintenance treatment for patients with high-risk neuroblastoma in China

Abstract Background High-risk neuroblastoma (HR-NB) is associated with high metastatic and relapse rates that require intensive multimodal treatment. We evaluated the efficacy and safety of dinutuximab beta as first-line maintenance immunotherapy in pediatric patients with HR-NB in real-world clinic...

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Main Authors: Xuedi Yu, Suyi Kang, Junjie Ge, Jingfu Wang
Format: Article
Language:English
Published: BMC 2025-03-01
Series:BMC Pediatrics
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Online Access:https://doi.org/10.1186/s12887-025-05568-x
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author Xuedi Yu
Suyi Kang
Junjie Ge
Jingfu Wang
author_facet Xuedi Yu
Suyi Kang
Junjie Ge
Jingfu Wang
author_sort Xuedi Yu
collection DOAJ
description Abstract Background High-risk neuroblastoma (HR-NB) is associated with high metastatic and relapse rates that require intensive multimodal treatment. We evaluated the efficacy and safety of dinutuximab beta as first-line maintenance immunotherapy in pediatric patients with HR-NB in real-world clinical settings in China. Methods We retrospectively reviewed the clinical records of pediatric patients with newly diagnosed HR-NB in the hospital from October 2021 to November 2023. Patients treated with dinutuximab beta in combination with granulocyte-macrophage colony-stimulating factor (GM-CSF) and isotretinoin as the first-line maintenance therapy were included in this study. Among patients with residual disease after completing induction and consolidation treatment, those with partial response (PR) or very good partial response (VGPR) except for bone marrow (BM) residue were also administrated vincristine/irinotecan/temozolomide (VIT) chemotherapy. Results Fifty-one patients with newly diagnosed HR-NB who achieved at least PR before immunotherapy were evaluated. At the end of immunotherapy, the objective response rate (ORR) in 33 patients with evidence of disease was 60.6% (95% confidence interval (CI), 42.1-77.1%) and the complete response rate (CRR; n = 18) was 54.5% (95% CI, 36.4-71.9%). The 2-year event-free survival (EFS) rate and overall survival (OS) rate were 80.1% (95% CI, 66.2-88.8%) and 97.6% (95% CI, 84.3-99.7%), respectively. The 2-year EFS rate was higher in patients with CR (94.4%; 95% CI, 66.6-99.2%) than in non-CR patients (72.6%; 95% CI, 53.9-84.7%). Dinutuximab beta was well tolerated in patients and had fewer side effects, which decreased over time. Co-treatment of dinutuximab beta with VIT chemotherapy did not require discontinuation in patients undergoing immunochemotherapy. Conclusion The study showed promising efficacy and safety of dinutuximab beta as the first-line maintenance immunotherapy for pediatric patients with HR-NB. Notably, the combination of dinutuximab beta with GM-CSF and VIT chemotherapy could be used for treating patients who did not achieve CR after previous multimodal therapy.
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spelling doaj-art-e7ce0f18581647c7bde00d51005a7dc02025-08-20T02:52:19ZengBMCBMC Pediatrics1471-24312025-03-0125111110.1186/s12887-025-05568-xA clinical observational study of dinutuximab beta as first-line maintenance treatment for patients with high-risk neuroblastoma in ChinaXuedi Yu0Suyi Kang1Junjie Ge2Jingfu Wang3Department of Pediatric Oncology, Shandong Cancer Hospital and Institute, Shandong First Medical University and Shandong Academy of Medical SciencesDepartment of Pediatric Oncology, Shandong Cancer Hospital and Institute, Shandong First Medical University and Shandong Academy of Medical SciencesDepartment of Pediatric Oncology, Shandong Cancer Hospital and Institute, Shandong First Medical University and Shandong Academy of Medical SciencesDepartment of Pediatric Oncology, Shandong Cancer Hospital and Institute, Shandong First Medical University and Shandong Academy of Medical SciencesAbstract Background High-risk neuroblastoma (HR-NB) is associated with high metastatic and relapse rates that require intensive multimodal treatment. We evaluated the efficacy and safety of dinutuximab beta as first-line maintenance immunotherapy in pediatric patients with HR-NB in real-world clinical settings in China. Methods We retrospectively reviewed the clinical records of pediatric patients with newly diagnosed HR-NB in the hospital from October 2021 to November 2023. Patients treated with dinutuximab beta in combination with granulocyte-macrophage colony-stimulating factor (GM-CSF) and isotretinoin as the first-line maintenance therapy were included in this study. Among patients with residual disease after completing induction and consolidation treatment, those with partial response (PR) or very good partial response (VGPR) except for bone marrow (BM) residue were also administrated vincristine/irinotecan/temozolomide (VIT) chemotherapy. Results Fifty-one patients with newly diagnosed HR-NB who achieved at least PR before immunotherapy were evaluated. At the end of immunotherapy, the objective response rate (ORR) in 33 patients with evidence of disease was 60.6% (95% confidence interval (CI), 42.1-77.1%) and the complete response rate (CRR; n = 18) was 54.5% (95% CI, 36.4-71.9%). The 2-year event-free survival (EFS) rate and overall survival (OS) rate were 80.1% (95% CI, 66.2-88.8%) and 97.6% (95% CI, 84.3-99.7%), respectively. The 2-year EFS rate was higher in patients with CR (94.4%; 95% CI, 66.6-99.2%) than in non-CR patients (72.6%; 95% CI, 53.9-84.7%). Dinutuximab beta was well tolerated in patients and had fewer side effects, which decreased over time. Co-treatment of dinutuximab beta with VIT chemotherapy did not require discontinuation in patients undergoing immunochemotherapy. Conclusion The study showed promising efficacy and safety of dinutuximab beta as the first-line maintenance immunotherapy for pediatric patients with HR-NB. Notably, the combination of dinutuximab beta with GM-CSF and VIT chemotherapy could be used for treating patients who did not achieve CR after previous multimodal therapy.https://doi.org/10.1186/s12887-025-05568-xHigh-risk neuroblastomaDinutuximab betaGD2-antibody immunotherapyGM-CSFFirst-line maintenanceChemotherapy
spellingShingle Xuedi Yu
Suyi Kang
Junjie Ge
Jingfu Wang
A clinical observational study of dinutuximab beta as first-line maintenance treatment for patients with high-risk neuroblastoma in China
BMC Pediatrics
High-risk neuroblastoma
Dinutuximab beta
GD2-antibody immunotherapy
GM-CSF
First-line maintenance
Chemotherapy
title A clinical observational study of dinutuximab beta as first-line maintenance treatment for patients with high-risk neuroblastoma in China
title_full A clinical observational study of dinutuximab beta as first-line maintenance treatment for patients with high-risk neuroblastoma in China
title_fullStr A clinical observational study of dinutuximab beta as first-line maintenance treatment for patients with high-risk neuroblastoma in China
title_full_unstemmed A clinical observational study of dinutuximab beta as first-line maintenance treatment for patients with high-risk neuroblastoma in China
title_short A clinical observational study of dinutuximab beta as first-line maintenance treatment for patients with high-risk neuroblastoma in China
title_sort clinical observational study of dinutuximab beta as first line maintenance treatment for patients with high risk neuroblastoma in china
topic High-risk neuroblastoma
Dinutuximab beta
GD2-antibody immunotherapy
GM-CSF
First-line maintenance
Chemotherapy
url https://doi.org/10.1186/s12887-025-05568-x
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