难治性癫痫相关脑肿瘤 36 例临床病理学特征分析

摘要： 目的 分析并比较因难治性癫痫接受神经外科手术的 36 例脑肿瘤患者的临床病理学特点。方法 对 2008—2014 年在北京市海淀医院功能神经科接受癫痫神经外科手术治疗的 36 例脑肿瘤患者的临床资料及病理资料进行回顾性分析。结果 36 例患者中男 18 例， 女 18 例， 发病年龄 1~37 岁， 平均 （14.05±1.67） 岁， 病程 2~27 年， 平均 （10.04±1.19） 年。切除脑肿瘤标本病理诊断为神经节细胞胶质瘤 （GG） 13 例 （36.1%， WHOⅠ级 12 例， Ⅱ级 1 例），胚胎发育不良性神经上皮瘤（DNT） 2 例（5.5%， WHOⅠ级）， 具有过渡特点的混合性神经元-胶质病变/肿瘤 5 例（13.9%）， 血管中心性胶质瘤（AG） 1 例（2.8%， WHOⅠ级）， 多形性黄色星形细胞瘤（PXA） 1 例（2.8%， WHOⅡ级）， 星形细胞瘤 4 例（11.1%， WHOⅡ级）， 少突星形细胞瘤 3 例（8.3%， WHOⅠ~Ⅱ级 2 例， WHOⅡ级 1 例）， 少突胶质细胞瘤 2 例（5.5%， WHOⅠ~Ⅱ级 1 例， WHOⅡ级 1 例）， 海绵状血管瘤 4 例（11.1%）， Sturge-Weber 综合征（SWS） 1 例（2.8%）。同时伴发皮质发育不良 （FCD） 者 25 例 （69.4%）。术后随访 0.5~7 年， EngelⅠ级 28 例 （77.8%）， EngelⅡ级 4 例 （11.1%）， EngelⅢ级 2 例 （5.6%）， EngelⅣ级 1 例 （2.8%）， 失访 1 例 （2.8%）。肿瘤发生在颞叶者 25 例 （69.4%）。结论 难治性癫痫相关的脑肿瘤常见为低级别混合性神经元-胶质肿瘤， 其中 GG 最多。大多脑肿瘤周围伴发 FCDⅢb， FCD 的形成与脑肿瘤相关， 是癫痫发生的原因。脑肿瘤病理类型很可能与手术预后相关。

Brain tumors in patients with intractable epilepsy: a clinicopathologic study of thirty-six cases

Abstract： Objective To study the clinicopathologic features of brain tumors in patients with medically intractable lepsy. Methods The clinical, radiologic and pathologic features of brain tumors in thirty-six patients with intractable lepsy encountered during the period from 2008 to 2014 in the Epilepsy Center of Haidian Hospital were retrospectively iewed. Results There were 18 males and 18 females in thirty-six patients. The mean age of seizure onset and disease ation were (14.05 ± 1.67) years and (10.04 ± 1.19) years respectively. The histological types of brain tumors included glioglioma (12/36, WHO gradeⅠ， 1/36, WHO gradeⅡ), dysembryeplastic neuroepithelial tumor (2/36, WHO grade Ⅰ), omorphic xanthoastrocytoma (1/36, WHO grade Ⅱ), angiocentric glioma (1/36, WHO gradeⅠ), astrocytoma (4/36, WHO de Ⅱ), oligoastrocytoma (1/36, WHO grade Ⅱ, 2/36, WHO grade Ⅰ-Ⅱ), oligodendroglioma (1/36, WHO gradeⅠ-Ⅱ， 1/36, HO grade Ⅱ), cavernous hemangioma (4/36) and Sturge-Weber syndrome (1/36). Most of these tumors were located intemporal lobe (25/36, 69.4%). Patients were followed up for 0.5-7 years after operation. One patient was lost for follow up. Seizure outcome after the epilepsy operation revealed that 28 patients (77.8%) had Engel gradeⅠ, 4 patients (11.1%) had Engel gradeⅡ， 2 patients (5.6%) had Engel grade Ⅲ， 1 patient (2.8%) had Engel grade Ⅳ. Conclusion Brain tumors in patients with medically intractable epilepsy are almost low grade tumors of the nervous system. Focal cortical dysplasia is existed in most brain tissues from the epilepsy operation. Low grade tumors of the nervous system have close relation with focal cortical dysplasia in patients with medically intractable epilepsy. It is possible that the classifications of pathology diagnosis has connection with prognosis.