Devaux B, Chassoux F, Landré E, Turak B, Laurent A, Zanello M, Mellerio C, Varlet P
Service de neurochirurgie, hôpital Sainte-Anne, université Paris Descartes, 1, rue Cabanis, 75014 Paris, France.
Service de neurochirurgie, hôpital Sainte-Anne, université Paris Descartes, 1, rue Cabanis, 75014 Paris, France.
Neurochirurgie. 2017 Jun;63(3):227-234. doi: 10.1016/j.neuchi.2016.10.009. Epub 2017 May 12.
Dysembryoplastic neuroepithelial tumors and gangliogliomas are developmental glioneuronal tumors usually revealed by partial epilepsy. High epileptogenicity, childhood epilepsy onset, drug-resistance, temporal location, and seizure freedom after complete resection are common characteristics of both tumors. We report the specificity of surgical management, functional results and seizure outcome in cases of a tumor location in eloquent areas.
Among 150 patients (88 males, 3-55 years) operated on for refractory epilepsy due to a glioneuronal tumor (1990-2015), 30 (20%, dysembryoplastic neuroepithelial tumors=21; gangliogliomas=9) had a tumor located in an eloquent cortex (sensory-motor, insular or language areas). Surgery was performed after a preoperative work-up, including stereo-electroencephalography in 48 patients (26%) and functional MRI in 100 (67%). MRI-guided lesionectomy was mainly performed in extra-temporal location, whereas an additional corticectomy was performed in a temporal location. Tumor microsurgical resections were guided using neuronavigation and cortical/subcortical electrical stimulations. Multiple stereotactic thermocoagulations were performed in two insular tumors.
New motor/language deficits related to eloquent areas occurred postoperatively in 6/30 patients (20%) without any major permanent disability. Minor sensorimotor (n=2) and moderate language disturbance (n=1) persisted in three of them. Postoperative seizure-free outcome (mean follow-up>5 years) was obtained in 81% of the entire series, but significantly decreased to 60% in eloquent areas. Incomplete tumor resection was the main cause of surgical failure. However, unfavorable seizure outcome was also observed despite complete tumor resection. Malignant transformation occurred in one ganglioglioma.
Epilepsy surgery for benign glioneuronal tumors in eloquent areas provides acceptable results regarding the functional risks. Complete tumor resection is crucial for long-term favorable outcome.
胚胎发育不良性神经上皮肿瘤和神经节细胞胶质瘤是发育性神经胶质神经元肿瘤，通常由部分性癫痫引发。高致痫性、儿童期癫痫发作、耐药性、颞叶定位以及完全切除后癫痫发作缓解是这两种肿瘤的共同特征。我们报告了肿瘤位于功能区的病例中手术治疗的特异性、功能结果和癫痫发作结局。
在1990年至2015年因神经胶质神经元肿瘤接受难治性癫痫手术的150例患者（88例男性，年龄3至55岁）中，30例（20%，胚胎发育不良性神经上皮肿瘤21例；神经节细胞胶质瘤9例）肿瘤位于功能皮层（感觉运动区、岛叶或语言区）。术前进行了包括48例（26%）立体定向脑电图和100例（67%）功能磁共振成像在内的检查后进行手术。磁共振成像引导下的病灶切除术主要在颞外部位进行，而在颞叶部位则需额外进行皮质切除术。肿瘤显微手术切除采用神经导航和皮质/皮质下电刺激引导。对两例岛叶肿瘤进行了多次立体定向热凝治疗。
6/30例患者（20%）术后出现与功能区相关的新的运动/语言功能缺损，但无任何严重永久性残疾。其中3例患者仍存在轻微感觉运动功能障碍（n = 2）和中度语言障碍（n = 1）。整个系列中81%的患者术后达到无癫痫发作结局（平均随访>5年），但在功能区显著降至60%。肿瘤切除不完全是手术失败的主要原因。然而，尽管肿瘤完全切除，仍观察到不良的癫痫发作结局。1例神经节细胞胶质瘤发生了恶性转化。
功能区良性神经胶质神经元肿瘤的癫痫手术在功能风险方面提供了可接受的结果。肿瘤完全切除对于长期良好结局至关重要。
