Kumar Narendra, Madan Renu, Dracham Chinna Babu, Khosla Divya, Das Namrata, Dey Treshita, Elangovan Arun, Tripathi Manjul, Gupta Kirti, Ahuja Chirag K, Kapoor Rakesh
Department of Radiotherapy & Oncology, PGIMER, Chandigarh, India.
Department of Radiation Oncology, Queen's NRI Hospital, Visakhapatnam, India.
Clin Neurol Neurosurg. 2021 Mar;202:106481. doi: 10.1016/j.clineuro.2021.106481. Epub 2021 Jan 13.
Intracranial germ cell tumors (GCTs) are relatively rare neoplasms, representing 2-3 % of paediatric brain tumors in Western countries and 8-15 % in East Asia. Here, we discuss the clinical features and treatment outcomes in patients with central nervous system (CNS) GCTs treated at our institute.
Medical records of all primary CNS GCT patients were retrieved retrospectively from 2007 to 2019. Demographic, clinical, treatment and follow up details were entered in a predesigned proforma. Overall survival (OS) and progression-free survival was computed using Kaplan-Meier method and Log-Rank test. Effect of various prognostic factors on survival outcomes was assessed by univariate and multivariate analysis.
A total of 28 CNS GCT patients were included in this analysis. Median age at presentation was 17 years (range, 7-45 years) with a male to female ratio of 1.8:1. Pineal region was the commonest location, encountered in 15 patients (53.6 %). Pure germinoma was the most frequent histology observed, seen in 19 patients (67.9 %). Male gender and germinoma histology were highly associated with pineal location (p = 0.043 and p = 0.052, respectively). Fourteen patients underwent surgical intervention and nine patients underwent biopsy for diagnostic purpose or to relieve the obstructive symptoms. Only 23 patients (82.1 %) received chemotherapy. However, all patients received radiotherapy (Craniospinal irradiation/whole brain radiotherapy/whole ventricular radiotherapy/ or local radiotherapy). After a median follow-up of 53 months (range, 7-150), 23 patients (82.1 %) were alive. OS was significantly affected by histology (89 % in germinoma vs. 60 % in non-germinomatous, p = 0.054) and location (93 % in pineal region vs. 64.2 % in other location, p = 0.042). Age, gender and surgery did not have any impact on the survival outcomes.
CNS GCTs are relatively rare and heterogeneous neoplasms commonly seen in pineal and suprasellar locations. A combination of chemotherapy and radiotherapy had shown excellent outcomes.
颅内生殖细胞肿瘤（GCTs）是相对罕见的肿瘤，在西方国家占儿童脑肿瘤的2%-3%，在东亚占8%-15%。在此，我们讨论在我院接受治疗的中枢神经系统（CNS）GCTs患者的临床特征和治疗结果。
回顾性检索2007年至2019年所有原发性CNS GCT患者的病历。将人口统计学、临床、治疗和随访细节录入预先设计的表格。采用Kaplan-Meier法和Log-Rank检验计算总生存期（OS）和无进展生存期。通过单因素和多因素分析评估各种预后因素对生存结果的影响。
本分析共纳入28例CNS GCT患者。发病时的中位年龄为17岁（范围7-45岁），男女比例为1.8:1。松果体区是最常见的部位，15例患者（53.6%）出现该部位病变。纯生殖细胞瘤是最常见的组织学类型，19例患者（67.9%）为该类型。男性性别和生殖细胞瘤组织学与松果体区位置高度相关（分别为p = 0.043和p = 0.052）。14例患者接受了手术干预，9例患者接受了活检以明确诊断或缓解梗阻症状。只有23例患者（82.1%）接受了化疗。然而，所有患者均接受了放疗（全脑脊髓照射/全脑放疗/全脑室放疗/或局部放疗）。中位随访53个月（范围7-150个月）后，23例患者（82.1%）存活。组织学类型（生殖细胞瘤为89%，非生殖细胞瘤为60%，p = 0.054）和部位（松果体区为93%，其他部位为64.2%，p = 0.042）对OS有显著影响。年龄、性别和手术对生存结果无任何影响。
CNS GCTs是相对罕见且异质性的肿瘤，常见于松果体区和鞍上部位。化疗和放疗联合应用显示出良好的效果。
