儿童颅内生殖细胞肿瘤的临床特点和预后分析

Clinical characteristics and prognosis analysis of intracranial germ cell tumors in children

目的总结儿童颅内生殖细胞肿瘤(IGCTs)的临床特点和预后.方法回顾性分析2015年5月至2022年4月首都医科大学附属北京儿童医院神经外科收治的38例儿童IGCTs患者的临床资料.38例患儿中,生殖细胞瘤8例,非生殖细胞瘤性生殖细胞肿瘤(NGGCTs)30例.其中34例经开颅手术切除确诊,2例经病变活组织检查确诊,1例经诊断性化疗诊断为生殖细胞瘤,1例结合血清肿瘤标志物和影像学检查诊断为NGGCTs.采用Kaplan-Meier法和log-rank分析探讨儿童IGCTs患者预后的影响因素.结果38例患儿中,男女比例为4.4∶1.0;中位年龄(范围)为9.3岁(0.9~14.0岁),年龄集中在2~4岁和8~14岁.肿瘤的位置以松果体区(65.8%,25/38)和鞍区(10.5%,4/38)常见.松果体区IGCTs最常见的表现为颅高压症状(80.0%,20/25),鞍区IGCTs最常见的表现为多饮、多尿(3/4).38例患儿的中位随访时间(范围)为40.3个月(0.6~86.7个月).至末次随访,存活31例,死亡5例,失访2例.总体5年生存率为90.2%(除外非肿瘤因素的死亡),生殖细胞瘤患儿的5年无进展生存率和总生存率均为83.3%,NGGCTs患儿分别为83.8%和91.9%.与年龄>6岁的患儿相比,年龄≤6岁的患儿中NGGCTs更常见(12/12对比18/26,P=0.039).结论儿童IGCTs的发病年龄呈双峰分布,病变多见于松果体区和鞍区.患儿的临床表现与其发病部位有关,总体生存预后较好.

Objective To summarize the clinical characteristics and prognosis of intracranial germ cell tumors(IGCTs)in children.Methods The clinical data of 38 pediatric patients with IGCTs admitted to the Neurosurgery Department of Beijing Children's Hospital,Capital Medical University from May 2015 to April 2022 were retrospectively analyzed.Among the 38 children,8 had germinoma and 30 had non-germinomatous germ cell tumors(NGGCTs).Among them,34 cases were confirmed by craniotomy,2 cases were confirmed by lesion biopsy,1 case was diagnosed as germinoma by diagnostic chemotherapy,and 1 case was diagnosed as NGGCT by combining tumor markers and imaging examinations.The Kaplan-Meier method and log-rank analysis were used to explore the influencing factors on the outcomes of children with IGCTs.Results Among the 38 children,the male to female ratio was 4.4:1.0;the age[M(range)]was 9.3(0.9-14.0)years old,and the ages were concentrated in 2 to 4 years old and 8 to 14 years old.The most common locations of tumors were the pineal region(65.8%,25/38)and sellar region(10.5%,4/38).The most common manifestation of IGCTs in the pineal region was intracranial hypertension(80.0%,20/25),and the most common manifestation of IGCTs in the sellar region was polydipsia and polyuria(3/4).The median follow-up time(range)of 38 children was 40.3 months(0.6-86.7 months).As of the last follow-up,31 cases were alive,5 cases had died,and 2 cases were lost to follow-up.The overall 5-year survival rate was 90.2%(excluding death from non-tumor factors),the 5-year progression-free survival rate and overall survival rate of children with germinoma were 83.3%,and the 5-year progression-free survival rate and overall survival rate of children with NGGCTs were 83.8%and 91.9%respectively.Compared with children aged>6 years,NGGCTs were more common in children aged≤6 years(12/12 vs.18/26,P=0.039).Conclusions The age of onset of IGCTs in children shows a bimodal distribution,and lesions are more common in the pineal and sellar regions.The clinical manifestations of children are related to the site of lesion,and the survival prognosis is good.