WANG Caihong 1ZHAO Wenying 1HAN Dandan 1LEI Yang 1LI Yun 1LIU Zhaohe2
作者信息
- 1. Department of Pathology,Shanxi Children's Hospital,Taiyuan 030013
- 2. Department of Neurosurgery,Shanxi Children's Hospital,Taiyuan 030013,China
- 折叠
摘要
Abstract
Objective:Pediatric posterior fossa ependymomas exhibit marked clinical heterogeneity and widely varied prognoses.Molecular subtyping plays an increasingly important role in prognostic evaluation;however,its associations with clinicopathological features and outcomes require further clarification.This study aims to investigate the clinicopathological characteristics,molecular subtypes,and prognostic correlations of pediatric posterior fossa ependymomas,providing a reference for individualized clinical management.
Methods:Twenty-one children with posterior fossa ependymoma who underwent tumor resection at Shanxi Children's Hospital between August 2018 and August 2025 were included.Clinical data,pathological features,immunophenotype,molecular subtype,and survival outcomes were analyzed.
Results:Among the 21 patients,13 were male and 8 were female,aged 1 year 2 months to 14 years(median 3 years 7 months).Histology:All cases showed perivascular pseudorosettes;true ependymal rosettes/ependymal clefts or more complex structures were observed in some cases,and 3 tumors infiltrated the brain parenchyma.Immunophenotype:Tumor cells diffusely expressed glial fibrillary acidic protein(GFAP)and S-100 protein.Epithelial membrane antigen(EMA)and D2-40(podoplanin)showed paranuclear dot-like or luminal-border positivity.H3K27me3 nuclear positivity was detected in 5 cases,and EZH inhibitory protein(EZHIP)nuclear positivity in 16 cases.Histological grading:WHO grade 2 in 15 cases(71.4%)and WHO grade 3 in 6 cases(28.6%).Molecular subtyping:Posterior fossa ependymoma type A(PFA)in 16 cases(76.2%)and type B(PFB)in 5 cases(23.8%).Gross-total resection was achieved in 13 cases(61.9%)and subtotal resection in 8 cases(38.1%).The median overall survival was 37 months,and the 5-year overall survival rate was 42.9%.Univariate analysis(log-rank test)showed that median OS was significantly associated with molecular subtype(PFA vs PFB),extent of resection(gross-total vs subtotal),and WHO grade(grade 2 vs grade 3),with statistically significant differences(P=0.012,P=0.025,and P=0.038,respectively).
Conclusion:PFA is the predominant subtype among pediatric posterior fossa ependymoma.PFA,WHO grade 3,and subtotal resection are adverse prognostic factors.Comprehensive therapeutic strategies should be formulated based on molecular subtype,extent of tumor resection,and WHO grade.关键词
儿童/后颅窝/室管膜瘤/临床病理特征/分子分型/预后Key words
children/posterior fossa/ependymoma/clinicopathologic characteristics/molecular subtyping/prognosis
