ArticleBMC cancer2026
Clinical analysis and prognostic factors in children with neuroblastoma-associated opsoclonus-myoclonus syndrome: a multicenter retrospective study.
Article in BMC cancer, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
backgroundThe long-term neurological outcomes for children diagnosed with neuroblastoma associated with Opsoclonus Myoclonus Syndrome (NB-OMS) are not well characterized, primarily due to the infrequency of the condition. This multicenter study sought to evaluate the rate of neurological recovery and the long-term neurodevelopmental sequelae within this patient population.
methodsA retrospective study at nine Chinese medical centers (January 2015 - January 2025) involved 39 children with NB-OMS. Researchers gathered data on demographics, clinical presentation, tumor traits, treatment, and outcomes. Non-parametric tests and multivariate logistic regression identified prognostic factors.
resultsIn this study, a cohort of 39 patients was examined, with a median age at onset of 22 months. The retroperitoneum was identified as the most prevalent tumor location, accounting for 79.5% of cases. The majority of tumors were classified within the low-risk and intermediate risk, as determined by the International Neuroblastoma Risk Group Staging System, comprising 87.2% of the sample. Follow-up was conducted for 37 patients, all of whom exhibited symptom improvement, resulting in a 100% survival rate. Nonetheless, neurological sequelae were detected in 16 children (43.2%). A higher OMS severity score at onset demonstrated a strong trend towards significance (OR = 1.42, 95% CI: 1.00-2.01, P = 0.051). Multivariate analysis revealed that delayed initiation of treatment, defined as greater than 30 days, was a significant independent risk factor for the development of sequelae (Odds Ratio [OR] = 22.98, 95% Confidence Interval [CI]: 2.67-197.63, P = 0.004). Additionally, relapse of opsoclonus-myoclonus syndrome emerged as a significant independent risk factor (OR = 13.74, 95% CI: 1.37-137.34, P = 0.026). Furthermore, the use of combination immunotherapy, consisting of corticosteroids and intravenous immunoglobulin (IVIG), was associated with a significantly reduced median time to symptom resolution compared to non-combination therapy (5 months versus 8.5 months, P = 0.001).
conclusionOMS is a chronic immune disorder often misdiagnosed, leading to treatment delays. Prompt first-line immunotherapy can quickly alleviate OMS symptoms. Early treatment and reducing relapses are crucial for better long-term outcomes. Future multicenter studies are needed to confirm the effectiveness of new immunotherapies.
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