University Children's Hospital, Martin-Luther-University Halle-Wittenberg, Ernst-Grube-Str. 40, 06120 Halle, Germany.
Neuro Oncol. 2011 Jun;13(6):680-9. doi: 10.1093/neuonc/nor045.
Pediatric high-grade gliomas (HGGs) of the thalamic region account for up to 13% of pediatric HGGs and usually result in only anecdotal long-term survival. Because very little is known about these tumors, we aimed to further characterize them. In our series of 99 pediatric thalamic HGGs, there were no significant differences in survival between patients with tumors affecting the thalamus alone (including bithalamic lesions) and patients with tumors affecting the thalamus plus adjacent structures. Tumor resection (event-free survival/overall survival) and an early treatment response to radiotherapy/chemotherapy (event-free survival) had independent prognostic significance, as shown by Kaplan-Meier and multivariate Cox regression analyses. When we compared clinical characteristics and outcomes of pediatric thalamic HGG with those of pediatric (nonthalamic) supratentorial (n = 177) as well as pediatric pontine HGG (including diffuse intrinsic pontine gliomas; n = 234), we found that thalamic HGG shared more similarities with pontine than with supratentorial HGG, but overall, it appeared to represent a clinically distinct subgroup of pediatric HGG. The varying extent of tumor resection in the different tumor localizations may play some role in the observed clinical differences, as shown by multivariate Cox regression analyses, but the tumor site itself was also identified as an independent prognostic parameter. Thus, an additional location-specific effect on survival and/or tumor biology, despite different neurosurgical accessibility, has to be considered. Therefore, future investigations should try to further characterize the obviously site-specific heterogeneity of pediatric HGG on a molecular genetic basis.
儿童丘脑高级别胶质瘤（HGG）占儿童 HGG 的 13%，通常仅能获得一些长期生存的病例报告。由于对这些肿瘤知之甚少，我们旨在进一步对其进行特征描述。在我们的 99 例儿童丘脑 HGG 系列中，仅累及丘脑（包括双侧丘脑病变）的肿瘤患者与累及丘脑加相邻结构的肿瘤患者之间的生存没有显著差异。肿瘤切除术（无事件生存/总生存）和放疗/化疗早期治疗反应（无事件生存）通过 Kaplan-Meier 和多因素 Cox 回归分析具有独立的预后意义。当我们比较儿童丘脑 HGG 的临床特征和结局与儿童（非丘脑）幕上 HGG（n=177）和儿童桥脑 HGG（包括弥漫性内在桥脑胶质瘤；n=234）时，我们发现丘脑 HGG 与桥脑 HGG 的相似性多于与幕上 HGG 的相似性，但总体而言，它似乎代表了儿童 HGG 的一个独特临床亚组。不同肿瘤部位的肿瘤切除范围可能在观察到的临床差异中起一定作用，这通过多因素 Cox 回归分析得到证实，但肿瘤部位本身也被确定为独立的预后参数。因此，尽管神经外科的可及性不同，仍需要考虑特定部位对生存和/或肿瘤生物学的额外影响。因此，未来的研究应尝试在分子遗传学基础上进一步对明显的部位特异性儿童 HGG 异质性进行特征描述。
