Survival and prognostic factors of pediatric brainstem gliomas: a single institution experience of 96 cases

Abstract

Purpose Brainstem gliomas (BSGs) have a poor prognosis, especially in children. The clinical manifestations of pediatric brainstem gliomas (pBSGs) are atypical, and systematic studies in this population are scarce. This study aimed to investigate the comprehensive features of pBSGs and prognostic factors associated with survival. Methods Data from primarily diagnosed BSGs were collected, including clinical, radiological, treatment, and molecular characteristics. Survival analysis was performed by the Kaplan-Meier method and the Cox regression method. Results 96 BSG patients were included, and the median overall survival (OS) was 11.23 months. Primary symptoms included gait instability in 73 cases, choking on water in 49 cases, limb weakness in 48 cases, and personality changes in 27 cases. Univariate regression analysis showed that ring enhancement, Lansky score, H3K27M, TP53, and EZH2 protein expression might affect the survival of patients with BSG (P < 0.05). Multifactorial Cox regression analysis showed that the patients with H3K27M wild-type, Lansky score ≥ 60, and MRI without noticeable ring enhancement had a more extended survival period (P < 0.05). In patients with H3K27M mutation, the median OS of the EZH2 mutant patients was shorter (P = 0.025). Conclusion PBSGs have a poor prognosis and should be considered in the differential diagnosis of children who present clinically with unexplained behavioral changes. An accurate pathological biopsy is essential for prognosis.