Rajagopal Revathi, Leong Sheng Hoay, Jawin Vida, Foo Jen Chun, Ahmad Bahuri Nor Faizal, Mun Kein Seong, Azman Raja Rizal, Loh Jasmin, Yap Tsiao Yi, Ariffin Hany, Moreira Daniel C, Gottardo Nicholas G, Bouffet Eric, Ganesan Dharmendra
Department of Pediatrics, Division of Hematology-Oncology.
Department of Surgery, Division of Neurosurgery.
J Pediatr Hematol Oncol. 2021 Oct 1;43(7):e913-e923. doi: 10.1097/MPH.0000000000002116.
A higher incidence of pediatric intracranial germ cell tumors (iGCTs) in Asian countries compared with Western countries has been reported. In Malaysia, the literature regarding pediatric iGCTs have been nonexistent. The aim of this study was to review the management, survival, and long-term outcomes of pediatric iGCTs at a single tertiary center in Malaysia.
We retrospectively reviewed data from patients below 18 years of age with iGCTs treated at the University Malaya Medical Center (UMMC) from 1998 to 2017.
Thirty-four patients were identified, with a median follow-up of 3.54 years. Sixteen (47%) patients had pure germinoma tumors (PGs), and the remaining patients had nongerminomatous germ cell tumors (NGGCTs). The median age was 12 years, with a male:female ratio of 4.7:1. Abnormal vision, headache with vomiting, and diabetes insipidus were the commonest presenting symptoms. Twenty-eight patients received initial surgical interventions, 24 were treated with chemotherapy, and 28 received radiotherapy. Eight patients experienced relapses. The 5- and 10-year event-free survival rates were similar at 61.1%±12.6% and 42.9%±12.1% for PG and NGGCT, respectively. The 5- and 10-year overall survival rates were the same at 75.5%±10.8% and 53.3%±12.3% for PG and NGGCT, respectively. Four patients died of treatment-related toxicity. Most of the survivors experienced good quality of life with satisfactory neurologic status.
The survival rate of childhood iGCTs in UMMC was inferior to that reported in developed countries. Late diagnosis, poor adherence to treatment, and treatment-related complications were the contributing factors. Although these results highlight a single institution experience, they most likely reflect similar treatment patterns, outcomes, and challenges in other centers in Malaysia.
据报道，亚洲国家小儿颅内生殖细胞肿瘤（iGCTs）的发病率高于西方国家。在马来西亚，尚无关于小儿iGCTs的文献。本研究的目的是回顾马来西亚一家三级中心小儿iGCTs的治疗、生存情况及长期预后。
我们回顾性分析了1998年至2017年在马来亚大学医学中心（UMMC）接受治疗的18岁以下iGCTs患者的数据。
共纳入34例患者，中位随访时间为3.54年。16例（47%）患者为纯生殖细胞瘤（PGs），其余患者为非生殖细胞性生殖细胞肿瘤（NGGCTs）。中位年龄为12岁，男女比例为4.7:1。视力异常、头痛伴呕吐及尿崩症是最常见的首发症状。28例患者接受了初始手术干预，24例接受化疗，28例接受放疗。8例患者出现复发。PG和NGGCT的5年和10年无事件生存率分别相似，为61.1%±12.6%和42.9%±12.1%。PG和NGGCT的5年和10年总生存率分别相同，为75.5%±10.8%和53.3%±12.3%。4例患者死于治疗相关毒性。大多数幸存者生活质量良好，神经功能状态令人满意。
UMMC小儿iGCTs的生存率低于发达国家报道的水平。诊断延迟、治疗依从性差及治疗相关并发症是影响因素。尽管这些结果仅反映了单一机构的经验，但很可能反映了马来西亚其他中心类似的治疗模式、结果及挑战。
