Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, 101 Daehak-ro, Jongno-gu, Seoul, 03080, Republic of Korea.
Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Republic of Korea.
J Neurooncol. 2019 Jul;143(3):437-445. doi: 10.1007/s11060-019-03174-3. Epub 2019 May 3.
Dysembryoplastic neuroepithelial tumors (DNETs) are a common cause of chronic drug-resistant epilepsy and are known for their favorable surgical outcomes. Nevertheless, the seizure recurrence-free rate is not as favorable if tumorous nodules are present near the main mass. We call these small tumorous nodules in the vicinity of the main mass satellite lesions (SLs). We analyzed tumor and seizure control in the presence and following the subsequent removal of SLs.
We retrospectively reviewed the medical records, radiological data, and surgical procedures to obtain the outcomes of children who underwent resection surgery for DNET. The analyses were designed to address the associations among the demographic, tumor and seizure-related variables. A Cox proportional hazard model was used for the univariate and multivariate analyses.
In total, 39 consecutive patients were included (26 males and 13 females). SLs were found in 22 patients (56%). The year-to-year analysis of patients with Engel class I was approximately 80% during the follow-up period. However, the actual seizure recurrence-free survival (RFS) rate was 82, 73 and 70% at the first, second and fifth year, respectively. The patients who initially presented with SLs had 46% seizure recurrence rates, while those without SL had 18% seizure recurrence rates.
As the seizure-RFS rate significantly declines over time, a more accurate seizure-free rate analysis using survival curves could be important for determining the outcome of DNET surgery. A thorough review identifying satellite lesions preoperatively and using intraoperative neuronavigation, electrocorticography (ECoG) or intraoperative ultrasonography is warranted to accomplish the wide resection of tumors with accompanying satellite lesions.
胚胎发育不良性神经上皮肿瘤(DNET)是慢性耐药性癫痫的常见病因,其手术效果良好。然而,如果主病灶附近存在肿瘤结节,则其无癫痫发作的复发率并不理想。我们将这些主病灶附近的小肿瘤结节称为卫星病变(SLs)。我们分析了存在和切除 SLs 后肿瘤和癫痫的控制情况。
我们回顾性分析了病历、影像学资料和手术过程,以获取接受 DNET 切除术的儿童的治疗结果。分析旨在研究人口统计学、肿瘤和癫痫相关变量之间的关系。采用 Cox 比例风险模型进行单因素和多因素分析。
共纳入 39 例连续患者(男 26 例,女 13 例)。22 例患者(56%)发现有 SLs。在随访期间,Engel 分级为 I 级的患者逐年分析约为 80%。然而,实际的无癫痫发作复发率(RFS)分别为第 1、2 和 5 年的 82%、73%和 70%。最初表现为 SL 的患者癫痫复发率为 46%,而无 SL 的患者癫痫复发率为 18%。
由于癫痫发作 RFS 率随时间显著下降,使用生存曲线进行更准确的无癫痫发作率分析对于确定 DNET 手术结果可能很重要。术前仔细检查识别卫星病变,并在术中使用神经导航、皮层电图(ECoG)或术中超声有助于广泛切除肿瘤及其伴随的卫星病变。