Department of Neurosurgery, University Hospitals Leuven, Leuven, Belgium.
Department of Pediatric Neurology, University Hospitals Leuven, Leuven, Belgium.
Pediatr Neurosurg. 2021;56(4):317-327. doi: 10.1159/000515516. Epub 2021 May 7.
Tectal plate gliomas (TPG) constitute a distinct entity of benign tumors of the brain stem which show an indolent clinical course. Adequate treatment of secondary hydrocephalus is undoubtedly a major factor in the outcome. However, little is known about to what degree the tumor itself determines the long-term outcome of these patients.
We retrospectively analyzed and compared the clinical and radiological data of 16 pediatric TPG patients with data of 12 pediatric idiopathic aqueductal stenosis (IAS) patients treated in our center from 1988 to 2018. For both groups, we assessed the long-term outcome in terms of hydrocephalus management, and for the TPG group, we assessed tumor growth during follow-up. In a separate prospective part of the study, we performed a neuropsychological evaluation in a subgroup of patients using a standardized testing battery, covering intelligence, learning, memory, executive functions, and an inventory on depression.
In the TPG group, the mean clinical and radiological follow-up was 84 and 70 months, respectively. On average, the maximum diameter of the tumor increased by 11% (p = 0.031) and the estimated tumor volume with 35% (p = 0.026) on radiological follow-up. The fronto-occipital horn ratio (FOHR) decreased by 23% on average after treatment. In the IAS group, the mean clinical and radiological follow-up was 117 and 85 months, respectively. In this group, the FOHR decreased by 21% on average. Neurocognitive testing revealed significant higher scores in the TPG group on global intelligence (TPG = 109, IAS = 85.5, U = 3, p < 0.01, z = -2.71), performance (TPG= 100, IAS = 85, U = 7, p = 0.03, z = -2.2), and verbal intelligence (TPG = 122, IAS = 91.5, U = 2, p < 0.00, z = -2.87) as well as working memory (TPG = 109.5, IAS = 77, U = 0.5, p = 0.01, z = -2.46).
Our results suggest that the long-term outcome in TPG patients is acceptable and that cognition is substantially better preserved than in patients with IAS. This puts the idea of a significant contribution of the tumoral mass to disease outcome on the long term in question. Adequate and prompt management of hydrocephalus is the most important factor in long-term cognitive outcome.
顶盖胶质瘤（TPG）构成了脑干良性肿瘤的一个独特实体，其临床表现呈惰性。继发性脑积水的充分治疗无疑是决定这些患者预后的一个主要因素。然而，人们对肿瘤本身在多大程度上决定这些患者的长期预后知之甚少。
我们回顾性分析和比较了 16 例小儿 TPG 患者的临床和影像学数据，以及 1988 年至 2018 年在我们中心治疗的 12 例小儿特发性导水管狭窄（IAS）患者的数据。对于这两组患者，我们都评估了脑积水管理方面的长期预后，对于 TPG 组，我们还评估了随访期间的肿瘤生长情况。在研究的一个独立的前瞻性部分，我们使用标准化测试包对患者进行了神经心理学评估，涵盖了智力、学习、记忆、执行功能和抑郁量表。
在 TPG 组中，平均临床和影像学随访时间分别为 84 个月和 70 个月。平均而言，肿瘤最大直径增加了 11%（p = 0.031），肿瘤体积估计增加了 35%（p = 0.026）。治疗后，额枕角比（FOHR）平均下降了 23%。在 IAS 组中，平均临床和影像学随访时间分别为 117 个月和 85 个月。在这个组中，FOHR 平均下降了 21%。神经认知测试显示 TPG 组在整体智力（TPG=109，IAS=85.5，U=3，p<0.01，z=-2.71）、表现（TPG=100，IAS=85，U=7，p=0.03，z=-2.2）和言语智力（TPG=122，IAS=91.5，U=2，p<0.00，z=-2.87）以及工作记忆（TPG=109.5，IAS=77，U=0.5，p=0.01，z=-2.46）方面的评分明显更高。
我们的结果表明，TPG 患者的长期预后是可以接受的，并且认知功能明显比 IAS 患者保存得更好。这使得肿瘤对疾病长期预后有重大影响的观点受到质疑。充分和及时地治疗脑积水是长期认知预后的最重要因素。
