胚胎发育不良性神经上皮肿瘤的外科治疗方式及癫痫预后

Surgical strategy and seizure outcome in dysembryoplastic neuroepithelial tumor

目的探讨胚胎发育不良性神经上皮肿瘤（DNET）的外科治疗方式及癫痫预后。方法对手术治疗的18例DNET患者进行随访，其中4例失访；男10例，女4例；年龄4～25岁，平均10．3岁。所有患者均行单纯病变切除术，术中常规采用皮层脑电监测，部分患者采用术中超声确定肿瘤切除程度，癫痫发作情况采用Engel分级进行评判，并对临床资料、影像学资料进行总结分析。结果手术全切除12例，部分切除2例。随访24—96个月，平均46．6个月。术后EngelⅠ级12例，其中11例已停用抗癫痫药物，EngelⅡ级1例，Ⅳ级1例。癫痫预后较差者均为部分切除患者。结论肿瘤切除程度是影响DNET癫痫预后的主要影响因素。单纯病变切除辅以皮层电灼效果满意，术中超声可减少肿瘤残留，术中皮层脑电监测对癫痫预后有较大帮助。

Objective Dysembryoplastic neuroepithelial tumor （DNET） is a rare intracranial tumor with drug - resistant epilepsy. The authors present a review of 18 patients who underwent surgical removal of a DNET to discuss the surgical strategy epilepsy outcome. Methods There are 18 DNET patients from Aprial 2004 to Aprial 2010, 4 patients were lost to follow up. Medical records, neuroradiological images of all 14 patients were investigated. There were 10 male and 4 female patients. Age of patients ranged from 4 to 25 years, mean 10. 3. We preferred lesionectomy and intraoperative electrocorticography concomitantly. After tumor removal, we would coagulate surrounding cortex with bipolar if there were still spikes or sharp waves on electrocorticography. Engel prognosis was recorded for every patient. Results Time of follow - up ranged from 24 to 96 months, mean 46. 6 months. Tumor was removed totally in 12 cases, partially in 2 cases. Intraoperative ultrasound was used in five patients and found tumor omission in 1 among the 5 patients. Engel prognosis was I grade in 12 patients whose tumor was removed totally,and complete antiepileptic drugs withdrawn was achieved in 11 out of 12 patients. The other two patients got EngelII and IV prognosis respectively. Tumor residue didn＇t regrow in a follow - up time of 54,63 months respectively. Condusion Lesionectomy and bipolar coagulation are effective for seizure control. Residual tumor is a significant risk factor for poor seizure outcome. Intraoperative ultrasound are recommended to make sure total removal. Electrocorticography should be routinely used before and after tumor removal for a better seizure outcome.