Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Beijing, China; Pediatric Epilepsy Center, Peking University First Hospital, Beijing, China.
Pediatric Epilepsy Center, Peking University First Hospital, Beijing, China.
Pediatr Neurol. 2023 Dec;149:127-133. doi: 10.1016/j.pediatrneurol.2023.09.022. Epub 2023 Oct 2.
The postoperative epilepsy outcome and clinicopathological features in children with ganglioglioma (GG) are not well understood.
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.
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.
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.
儿童神经节细胞瘤（GG）术后癫痫的结局和临床病理特征尚不清楚。
收集了 51 例连续确诊为 GG 并接受手术的儿科患者的数据。分析了 CD34 表达与 BRAF V600E 突变与临床特征的相关性，并分析了影响癫痫结局的相关因素。
平均随访 44.2 个月，48 例患者无癫痫发作。GG 中 BRAF V600E 突变（78.8%）和 CD34 表达（77.8%）的比例较高。伴有 BRAF V600E 突变的癫痫发作年龄早于不伴有 BRAF V600E 突变的癫痫发作年龄。CD34 的表达随着发病年龄、癫痫持续时间和手术年龄的增加而增加。约 62.7%的患者存在局灶性皮质发育不良（FCD）I，11.8%的患者存在 GG 周围的 FCD II。癫痫的转归与 BRAF V600E 突变、CD34 表达以及与 FCD 的结合均无显著相关性。
儿童 GG 和癫痫的总体预后乐观，转归与 BRAF V600E 突变和 CD34（+）的存在并不密切相关。GG 周围的 FCD 可能为 I 型或 II 型。不完全切除周围 FCD 有癫痫控制不理想的风险。携带 BRAF V600E 突变的儿童可能更容易发生早发性癫痫。CD34 的表达更可能在年龄较大且癫痫持续时间较长的儿童中被检测到。
