Clinical characteristics and prognosis analysis of intracranial germ cell tumors in children
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.
Neoplasms,germ cell and embryonalChildDisease attributesPrognosisSurvival analysis
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