IS METHYLATION STATUS SUBGROUPING REALLY A STRONG PROGNOSTIC FACTOR IN PEDIATRIC POSTERIOR FOSSA EPENDYMOMA?

Abstract

The effective treatment of posterior fossa ependymomas is surgery followed by radio-chemotherapy. Our aim is to evaluate the effects of sex, age, methylation subgrouping, extent of resection, radiation treatment (RT), MIB-1 index, grade, ATRX and H3K27M mutations on prognosis in pediatric patients with posterior fossa ependymoma (PFE). This is a retrospective study. Forty-two children with PFE who had surgery in our institution between 1996 and 2018 were included. Formalin-fixed paraffin-embedded tumor samples were evaluated for H3K27me3 immunostaining, MIB-1 index, WHO grades, ATRX and H3K27M mutations.Samples with global H3K27me3 reduction were grouped as posterior fossa ependymoma group A (PFA), whereas tumor samples with H3K27me3 nuclear immunopositivity were grouped as posterior fossa ependymoma group B (PFB). Mean age of patients was 4.4 years (range 0.71-14.51). Thirty-one patients (73.8%) were PFA, whereas 11 patients (26.2%) were PFB. WHO grades of PFAs were statistically higher in comparison to WHO grades of PFBs. There are no significant differences between PFAs and PFBs in terms of resection rates, disease recurrence and survival parameters.Patients with total surgical excisions had significantly better PFS and OS rates. Extent of surgical excision is the most important prognostic indicator in PFEs. Prognostic effect of methylation subgrouping may be minimized with more aggressive surgical strategy in PFAs.