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Background <p>Neuroblastoma is the most common extracranial solid childhood tumour. Advances in risk stratification have improved survival by enabling better-tailored treatments. The Toronto Guidelines (TG) aim to standardise childhood cancer stage collection by cancer registries. However, population-based survival analyses by stage remain scarce. We aimed to study incidence and survival of neuroblastoma cases among Spanish children aged &lt; 15 years, with special attention to the role of stage at diagnosis.</p> Methods <p>The Spanish Registry of Childhood Tumours (RETI-SEHOP) and the Spanish Neuroblastoma Clinical Database (NBL-CDB) were cross-linked to achieve nearly complete registration of neuroblastoma in Spain for the period of diagnosis 2000–2021. Kaplan-Meier estimates were obtained for overall survival (OS). Adjusted survival was estimated using a Cox proportional hazards model with stratification by age group.</p> Results <p>During 2000–2021, 1774 neuroblastoma cases aged 0–14 years were recorded, making a rough average of 81 cases diagnosed annually. Age-standardised incidence rate (World) 2000–2021: 14.0 cases per million children (0–14 years). Distribution of stage at diagnosis remained stable from 2000 to 2021. 5-year OS increased from 74% (2000–2005) to 81% (2012–2017). Statistically significant OS differences were identified between sexes, age groups, tumour morphologies, primary sites and stages. Over time, survival improvement was centred mostly on male and locoregional cases.</p> Conclusions <p>Neuroblastoma incidence and survival results in Spain were consistent with those from other high-income countries. Survival improvements were related to changes in clinical management, such as administration of anti-GD2 immunotherapy or the start of the LINES clinical trial (EudraCT 2010-021396-81, registered on 15 June 2011; ClinicalTrials.gov identifier: NCT01728155). This study underscores the clinical interest in stage data being collected by cancer registries, as per the TG.</p>

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Incidence and survival among children with neuroblastoma in Spain over 22 years

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年10月10日

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  • Pau Alfonso-Comos,
  • Adela Cañete,
  • Álvaro Briz-Redón,
  • Elena Pardo Romaguera,
  • Vanessa Segura,
  • Désirée Ramal,
  • Blanca Martínez de las Heras,
  • Ana Fernández-Teijeiro

摘要

Background

Neuroblastoma is the most common extracranial solid childhood tumour. Advances in risk stratification have improved survival by enabling better-tailored treatments. The Toronto Guidelines (TG) aim to standardise childhood cancer stage collection by cancer registries. However, population-based survival analyses by stage remain scarce. We aimed to study incidence and survival of neuroblastoma cases among Spanish children aged < 15 years, with special attention to the role of stage at diagnosis.

Methods

The Spanish Registry of Childhood Tumours (RETI-SEHOP) and the Spanish Neuroblastoma Clinical Database (NBL-CDB) were cross-linked to achieve nearly complete registration of neuroblastoma in Spain for the period of diagnosis 2000–2021. Kaplan-Meier estimates were obtained for overall survival (OS). Adjusted survival was estimated using a Cox proportional hazards model with stratification by age group.

Results

During 2000–2021, 1774 neuroblastoma cases aged 0–14 years were recorded, making a rough average of 81 cases diagnosed annually. Age-standardised incidence rate (World) 2000–2021: 14.0 cases per million children (0–14 years). Distribution of stage at diagnosis remained stable from 2000 to 2021. 5-year OS increased from 74% (2000–2005) to 81% (2012–2017). Statistically significant OS differences were identified between sexes, age groups, tumour morphologies, primary sites and stages. Over time, survival improvement was centred mostly on male and locoregional cases.

Conclusions

Neuroblastoma incidence and survival results in Spain were consistent with those from other high-income countries. Survival improvements were related to changes in clinical management, such as administration of anti-GD2 immunotherapy or the start of the LINES clinical trial (EudraCT 2010-021396-81, registered on 15 June 2011; ClinicalTrials.gov identifier: NCT01728155). This study underscores the clinical interest in stage data being collected by cancer registries, as per the TG.