Department of Neurosciences, College of Medicine and Philippine General Hospital, University of the Philippines Manila, Manila, Philippines; Department of Clinical Epidemiology, College of Medicine, University of the Philippines Manila, Manila, Philippines.
Department of Neurosciences, Section of Pediatric Neurology, Asian Brain Institute, Asian Hospital Medical Center, Muntinlupa, Philippines; Neuroscience Department, College of Medicine, San Beda University, Manila, Philippines.
World Neurosurg. 2020 Jul;139:90-96. doi: 10.1016/j.wneu.2020.03.212. Epub 2020 Apr 13.
Congenital glioblastoma multiforme (cGBM) is an infrequent primary central nervous system tumor occurring within the first few months of life with a reported poor overall prognosis. Our objective was to describe our own clinical case of cGBM and review the literature of cGBM cases with prolonged survival.
We report a case of cGBM with prolonged survival at 4 years. A systematic review was conducted on cases of cGBM with long-term childhood survival. We searched online databases until August 2019 for relevant articles. The patient underwent an emergency right hemicraniectomy with excision and biopsy of the right cerebral hemisphere mass and insertion of a ventriculoperitoneal shunt. At present, she is a 52-month-old child with good speech and minimal left hemiparesis and able to ambulate, with a Functional Independence Measure for Children score of 109. Out of 160 articles screened, there were 10 articles included. A total of 15 patients, including the present case, were analyzed qualitatively. The age at presentation ranged from 30 weeks' gestation to 35 days. Most patients underwent surgical excision (n = 13, 86.7%) and adjuvant chemotherapy (n = 10, 66.7%). The reported range of survival of these patients was from 27 to 110 months.
Limited evidence from 15 cases of cGBM suggests that surgical excision and/or chemotherapy may prolong the survival of patients. Therefore, these interventions may be offered and performed to patients with cGBM on a case-by-case basis. Larger clinical studies or registry-based information are necessary to substantiate the implications of our review.
先天性多形性胶质母细胞瘤(cGBM)是一种罕见的原发性中枢神经系统肿瘤,发生于生命的头几个月,总体预后较差。我们的目的是描述我们自己的 cGBM 临床病例,并回顾文献中具有延长生存时间的 cGBM 病例。
我们报告了一例 cGBM 病例,其生存时间延长至 4 年。对具有长期儿童生存的 cGBM 病例进行了系统回顾。我们在线数据库中搜索了截至 2019 年 8 月的相关文章。患者接受了紧急右侧开颅术,切除并活检右侧大脑半球肿块,并插入脑室-腹腔分流管。目前,她是一个 52 个月大的孩子,言语良好,左侧偏瘫轻微,能够行走,儿童功能独立性测量评分为 109。在筛选的 160 篇文章中,有 10 篇文章被纳入。共分析了 15 例患者,包括本病例。患者的发病年龄为 30 周妊娠至 35 天。大多数患者接受了手术切除(n = 13,86.7%)和辅助化疗(n = 10,66.7%)。这些患者的报告生存时间范围为 27 至 110 个月。
15 例 cGBM 的有限证据表明,手术切除和/或化疗可能延长患者的生存时间。因此,可以根据具体情况向 cGBM 患者提供并进行这些干预。需要更大的临床研究或基于登记的信息来证实我们的综述的意义。