Jiao Yang, Wang Meng, Liu Xueyou, Wang Junkuan, Shou Yuwei, Sun Hongwei
Department of Neurosurgery, the First Affiliated Hospital of Zhengzhou University, Jianshe East Road No 1Henan Province, Zhengzhou, 450000, People's Republic of China.
Department of Pathology, the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China.
Childs Nerv Syst. 2022 Aug;38(8):1469-1477. doi: 10.1007/s00381-022-05522-7. Epub 2022 Apr 26.
Tumors involved with subventricular zone (SVZ) predicted an adverse prognosis had been well proved in adult glioblastoma (GBM). However, we still know less about its impact on children due to the rarity of pediatric glioblastoma (pGBM). We performed this retrospective study to better understand the clinical and prognostic features of pGBM involved with SVZ.
Fifty-two patients diagnosed with pGBM at our center between January 2011 and January 2021 were selected for review to demonstrate the characteristics of tumor contacting SVZ. Thirty patients who underwent concurrent chemoradiotherapy and adjuvant chemotherapy postoperatively were selected for survival analysis.
Of all the 52 patients, 21 were found to contact SVZ and 31 were not. The median PFS and OS in SVZ + patients were 5.2 and 8.9 months, respectively, whereas median PFS and OS were 11.9 and 17.9 months, respectively, in SVZ - patients. Multivariate analysis showed that involvement of SVZ was an independent prognostic factor for OS while focality at diagnosis was an independent prognostic factor for PFS. Tumors contacted with SVZ tend to have larger volumes, lower incidence of epilepsy, and lower total resect rate and they were more likely to originate from midline location. Age at diagnosis; gender; adjuvant therapy; focality at diagnosis; focality at relapse; mutational status of H3K27M, MGMT, IDH1, and IDH2; and expression of P53 and ATRX protein failed to characterize SVZ + patients.
Involvement of SVZ predicted worse OS in pGBM and it had some distinct clinical features in comparison with those that did not contact with SVZ. Multifocal tumor at diagnosis was related to a shorter PFS. We should make a further step to clarify its molecular features.
在成人胶质母细胞瘤(GBM)中,累及脑室下区(SVZ)的肿瘤预后不良已得到充分证实。然而,由于儿童胶质母细胞瘤(pGBM)罕见,我们对其在儿童中的影响仍知之甚少。我们进行了这项回顾性研究,以更好地了解累及SVZ的pGBM的临床和预后特征。
选取2011年1月至2021年1月期间在本中心诊断为pGBM的52例患者进行回顾性分析,以展示肿瘤与SVZ接触的特征。选取30例术后接受同步放化疗和辅助化疗的患者进行生存分析。
在所有52例患者中,21例被发现与SVZ接触,31例未接触。SVZ+患者的中位无进展生存期(PFS)和总生存期(OS)分别为5.2个月和8.9个月,而SVZ-患者的中位PFS和OS分别为11.9个月和17.9个月。多因素分析显示,SVZ受累是OS的独立预后因素,而诊断时的局灶性是PFS的独立预后因素。与SVZ接触的肿瘤往往体积更大,癫痫发生率更低,全切除率更低,且更可能起源于中线部位。诊断时的年龄、性别、辅助治疗、诊断时的局灶性、复发时的局灶性、H3K27M、MGMT、IDH1和IDH2的突变状态以及P53和ATRX蛋白的表达均无法区分SVZ+患者。
SVZ受累预示pGBM的OS更差,与未与SVZ接触的肿瘤相比具有一些独特的临床特征。诊断时的多灶性肿瘤与较短的PFS相关。我们应进一步阐明其分子特征。