Pediatric mortality from neurofibromatosis and malignant peripheral nerve sheath tumors in Brazil, 2008-2023: a 16-year nationwide analysis of persistent disparities.

Authors

Karina Munhoz de Paula Alves Coelho, José Guilherme Pickler, José Cândido Caldeira Xavier-Júnior, Francis Rossetti Pedack, Raquel Francine Liermann Garcia, Carlos Frederico Fronza, Bruna Louise Silva, Acir Alves Coelho Junior, Paulo Henrique Condeixa de França, Bárbara Sarni Sanches, Priscila Ferraz Franczak, Gustavo Rassier Isolan, Marcelo Gerardin Poirot Land, Rafael Roesler

Published in

Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery. Volume 42. Issue 1. Jul 24, 2026. Epub Jul 24, 2026.

Abstract

Neurofibromatosis (NF) comprises genetic tumor predisposition syndromes with multisystem involvement, yet pediatric mortality data remain scarce in middle-income settings. Malignant peripheral nerve sheath tumors (MPNST) represent the leading cause of premature death in this population. This study analyzed 16-year temporal trends and regional disparities in pediatric NF/MPNST mortality and hospitalizations in Brazil.
We conducted a nationwide ecological study of individuals aged 0-19 years from 2008 to 2023. Mortality data were obtained from the Mortality Information System (SIM) and hospital admissions from the Hospital Information System (SIH/SUS), with temporal trends assessed using Prais-Winsten regression. We calculated Age-Specific Mortality Rates (ASMRs) and assessed excess mortality risk using Standardized Mortality Ratios (SMR) to identify regional disparities.
A total of 177 pediatric deaths were identified, with MPNST accounting for 64.9% (N = 115) and NF for 35.1% (N = 62). SMR analysis revealed significant geographic inequalities. Children aged 0-9 in the South region faced a mortality risk double that of the reference population (SMR = 2.02; 95% CI 1.08-3.46; p = 0.010), whereas adolescents in the North region exhibited significantly lower-than-expected mortality. Despite global therapeutic advances, mortality rates in Brazil remained statistically stagnant across all age groups and conditions (p values were non-significant). Conversely, NF-related hospitalizations demonstrated an increasing trend (+ 2.98% annually for ages 0-9; + 1.94% for ages 10-19), while MPNST admissions remained stable.
Pediatric mortality from NF and MPNST in Brazil has remained unchanged for 16 years, contrasting with the rising trend in NF-related hospitalizations. This discrepancy, coupled with marked regional disparities, suggests persistent structural challenges in early diagnosis and equitable access to specialized oncology care. These findings highlight a critical need for national notification systems and improved therapeutic strategies for affected children and adolescents.

PMID:
42496737
Bibliographic data and abstract were imported from PubMed on 24 Jul 2026.

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