Epilepsy Outcome and Pathology Analysis for Ganglioglioma: A Series of 51 Pediatric Patients

Pediatr Neurol. 2023 Dec:149:127-133. doi: 10.1016/j.pediatrneurol.2023.09.022. Epub 2023 Oct 2.

Abstract

Background: The postoperative epilepsy outcome and clinicopathological features in children with ganglioglioma (GG) are not well understood.

Methods: Data from 51 consecutive pediatric patients diagnosed with GGs who underwent surgery were collected. The correlations between the expression of CD34 and BRAF V600E mutations and clinical features were analyzed. The related factors affecting the outcome of epilepsy were analyzed.

Results: The average follow-up was 44.2 months, and 48 patients were seizure-free. A high proportion of BRAF V600E mutation (78.8%) and CD34 expression (77.8%) was detected in GG. The onset age of epilepsy with the BRAF V600E mutation was earlier than that without. The expression of CD34 increased with the age of onset, the duration of epilepsy, and the age of operation. Focal cortical dysplasia (FCD) I was found in 62.7% of patients, and FCD II was found in 11.8% of patients approximately in the cortex surrounding GG. There was no significant correlation between the outcome of epilepsy and BRAF V600E mutation, CD34 expression, and combination with FCD.

Conclusions: The overall outcome of GG and epilepsy in children is optimistic, and the outcome is not closely related to the presence of BRAF V600E mutation and CD34 (+). The FCD surrounding GG could be type I or type II. Incomplete resection of the surrounding FCD has the risk of unsatisfactory control of epilepsy. Children with the BRAF V600E mutation may be prone to early-onset epilepsy. The expression of CD34 is more likely to be detected in children with older age and a long duration of epilepsy.

Keywords: BRAF V600E; CD 34; Epilepsy; Focal cortical dysplasia; Ganglioglioma.

MeSH terms

  • Brain Neoplasms* / complications
  • Brain Neoplasms* / genetics
  • Brain Neoplasms* / surgery
  • Cerebral Cortex
  • Child
  • Epilepsy* / genetics
  • Epilepsy* / surgery
  • Focal Cortical Dysplasia*
  • Ganglioglioma* / complications
  • Ganglioglioma* / genetics
  • Ganglioglioma* / surgery
  • Humans
  • Proto-Oncogene Proteins B-raf / genetics

Substances

  • Proto-Oncogene Proteins B-raf