Ternier Jessica, Wray Alison, Puget Stéphanie, Bodaert Nathalie, Zerah Michel, Sainte-Rose Christian
Department of Pediatric Neurosurgery, Hôpital Necker-Enfants Malades, Paris, France.
J Neurosurg. 2006 Jun;104(6 Suppl):369-76. doi: 10.3171/ped.2006.104.6.369.
The authors characterized the clinical course of tectal plate lesions in a group of pediatric patients to identify the prognostic factors at presentation that predict progression, in an attempt to differentiate tectal hamartomas from tumors.
A retrospective review was conducted of the management of tectal plate lesions in children since the advent of magnetic resonance (MR) imaging at the authors' hospital (1984-2003). The lesion volume seen on MR images, the clinical and radiological features at presentation, and the clinical course of the population were analyzed for correlations. Forty children with tectal lesions presented in the typical delayed fashion (mean 8.5 months) with symptoms referable to hydrocephalus (93%). Fourteen children whose tumors demonstrated radiological progression (enlargement, contrast enhancement, or cystic change) were treated surgically. Histologically, 80% of the surgically treated lesions were low grade (with the other 20% consisting of one dysplasia, one high-grade tumor, and one unidentified tumor). Five patients required a second operation and one required a third. One patient died of a high-grade astrocytoma after undergoing surgery and radiotherapy; the other 39 patients remain clinically stable. The only factor predictive of tumor enlargement was lesion volume at presentation (p = 0.002). Distribution analysis revealed three subgroups based on lesion volume (< 4, 4-10, and > 10 cm3), which correlated with the clinical course of the disease.
Children with tectal lesions should undergo contrast-enhanced MR imaging and volume assessment at the time of presentation. After hydrocephalus has been managed with endoscopic third ventriculostomy, these children require prolonged, close clinical and radiological surveillance. Lesions with a volume less than 4 cm3 were likely to be hamartomas and followed a predominantly benign course, with few atypical cases progressing. All large lesions, defined as having a volume greater than 10 cm3 at presentation, eventually required treatment, and all were histologically determined to be tumors. An argument is made for earlier treatment of larger lesions with the aim of improving outcome.
作者对一组儿科患者的顶盖病变临床病程进行了特征分析，以确定就诊时可预测病情进展的预后因素，试图将顶盖错构瘤与肿瘤区分开来。
对作者所在医院自磁共振成像（MR）问世以来（1984 - 2003年）儿童顶盖病变的治疗情况进行回顾性分析。分析MR图像上的病变体积、就诊时的临床和放射学特征以及该群体的临床病程之间的相关性。40例顶盖病变患儿以典型的延迟方式（平均8.5个月）就诊，症状多与脑积水有关（93%）。14例肿瘤出现放射学进展（增大、强化或囊性变）的患儿接受了手术治疗。组织学检查显示，80%接受手术治疗的病变为低级别（其余20%包括1例发育异常、1例高级别肿瘤和1例无法明确的肿瘤）。5例患者需要二次手术，1例需要三次手术。1例患者在接受手术和放疗后死于高级别星形细胞瘤；其余39例患者临床情况稳定。唯一可预测肿瘤增大的因素是就诊时的病变体积（p = 0.002）。分布分析显示，根据病变体积（<4、4 - 10和>10 cm³）可分为三个亚组，这与疾病的临床病程相关。
顶盖病变患儿就诊时应进行增强MR成像和体积评估。在通过内镜下第三脑室造瘘术处理脑积水后，这些患儿需要长期、密切的临床和放射学监测。体积小于4 cm³的病变可能是错构瘤，主要呈良性病程，很少有非典型病例进展。所有就诊时体积大于10 cm³的大病变最终都需要治疗，且组织学检查均确定为肿瘤。为改善预后，主张对较大病变尽早治疗。
