难治性癫痫相关脑肿瘤36例临床病理学特征分析

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

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。

Objective To study the clinicopathologic features of brain tumors in patients with medically intractable epilepsy. Methods The clinical, radiologic and pathologic features of brain tumors in thirty-six patients with intractable epilepsy encountered during the period from 2008 to 2014 in the Epilepsy Center of Haidian Hospital were retrospectively reviewed. Results There were 18 males and 18 females in thirty-six patients. The mean age of seizure onset and disease duration were （14.05 ± 1.67） years and （10.04 ± 1.19） years respectively. The histological types of brain tumors included ganglioglioma （12/36, WHO gradeⅠ,1/36, WHO gradeⅡ）, dysembryeplastic neuroepithelial tumor （2/36, WHO gradeⅠ）, pleomorphic xanthoastrocytoma （1/36, WHO gradeⅡ）, angiocentric glioma （1/36, WHO gradeⅠ）, astrocytoma （4/36, WHO gradeⅡ）, oligoastrocytoma （1/36, WHO gradeⅡ, 2/36, WHO gradeⅠ-Ⅱ）, oligodendroglioma （1/36, WHO gradeⅠ-Ⅱ,1/36, WHO grade Ⅱ）, cavernous hemangioma （4/36） and Sturge-Weber syndrome （1/36）. Most of these tumors were located in temporal 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 gradeⅢ,1 patient （2.8%） had Engel gradeⅣ. 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.