1Department of Neurosurgery, Sestre milosrdnice University Hospital Center, Zagreb, Croatia; 2Josip Juraj Strossmayer University of Osijek, Faculty of Medicine, Osijek, Croatia; 3Department of Anatomy, University of Applied Health Sciences, Zagreb, Croatia; 4Josip Juraj Strossmayer University of Osijek, School of Dental Medicine and Health, Osijek, Croatia; 5University of Applied Health Sciences, Zagreb, Croatia.
Acta Clin Croat. 2021 Dec;60(4):627-631. doi: 10.20471/acc.2021.60.04.09.
Dysembryoplastic neuroepithelial tumors (DNETs) are benign neoplasms classified in the category of glioneuronal tumors. The estimated incidence of DNETs is 0.03 100,000 person year with the age peak in a range between 10 and 14 years, and decreasing dramatically with increasing age. They are seldom diagnosed in persons above 20 years of age, being a cause of tumor-related intractable epilepsy that begins in childhood or adolescence. They have been proven to be the second most common type of epileptogenic tumors in pediatric population. These rare tumors cause chronic drug-resistant partial complex seizures with or without secondary generalization. Herein, we provide institutional case series of six adult patients with temporal lobe DNET presenting with complex partial seizures. Lesionectomy was performed with tumor resection in three patients. In another three, partial resection was performed, whereas tumor remnant was left intact to avoid possible basal ganglia damage. All patients were seizure free postoperatively. Lesionectomy alone in temporal lobe epilepsy was associated with less favorable outcome than anterior temporal lobectomy. Total tumor removal is considered a major prognostic factor in most studies.
胚胎发育不良性神经上皮肿瘤(DNETs)是一种良性肿瘤,属于神经胶质神经元肿瘤。DNETs 的估计发病率为 0.03/100,000 人年,发病年龄高峰在 10 至 14 岁之间,随着年龄的增长而显著下降。DNETs 在 20 岁以上人群中很少被诊断,是导致儿童或青少年起病的肿瘤相关性难治性癫痫的原因之一。DNETs 已被证明是儿童人群中第二常见的致痫性肿瘤类型。这些罕见的肿瘤引起慢性药物难治性部分复杂发作,伴或不伴继发全面性发作。在此,我们提供了 6 例成人颞叶 DNET 患者的机构病例系列,这些患者表现为复杂部分性发作。3 例患者行病变切除术,另 3 例患者行部分切除术,而肿瘤残余部分保持完整,以避免可能的基底节损伤。所有患者术后均无癫痫发作。单纯病变切除术在颞叶癫痫中的预后不如前颞叶切除术。在大多数研究中,肿瘤完全切除被认为是一个主要的预后因素。