儿童基底节生殖细胞瘤的早期诊断和治疗

Early diagnosis and treatment for basal ganglia germinomas in children

目的探讨儿童基底节生殖细胞瘤的早期诊断和治疗。方法 2006年11月至2017年1月本院收治的21例基底节生殖细胞瘤患儿,分析其临床特点、影像学和肿瘤标志物特征。结果基底节生殖细胞瘤常以男性为主,少年多发,以一侧肌力下降为主要临床症状,伴智力下降;CT显示基底节区占位并压迫脑室和同侧颞叶萎缩;MRI显示T1相呈点、片状等信号或囊变样不均匀混杂增强信号,T2相可见明确的占位征象及同侧颞叶萎缩,脑沟、池增宽;生化检查HCG升高;诊断性放疗可以明显缩小瘤体,结合放化疗可以达到完全治愈的目的。结论影像学显示无明显边界的基底节区占位并同侧颞叶萎缩,11岁左右的男性,一侧肌力下降伴智力下降,且HCG异常升高,可初步诊断为基底节生殖细胞瘤,予行诊断性放疗明确诊断,完成化疗和放疗,减少手术风险。

Objective To study the early diagnosis and treatment for basal ganglia germinomas in children.Methods A total of 21 cases of basal ganglia germinomas(BGG)in children were analyzed according to clinical characteristics,neuroimaging data and tumor markers from November 2006 to January2017.Results BGG was characterized in male adolescents with progressive hemiparesis,intelligent deficiency and high level of serum HCG.The basal ganglia space-occupying lesion oppressed the ventricle with ipsilateral temporal lobe atrophy in brain CT.Tumor parenchyma was shown with isointense or heterogeneous enhancement on MRI T1 weighted images;T2 weighted images showed obvious local occupying effect and the atrophy of temporal lobe with widening of cerebral sulci and cisterns.After diagnostic radiotherapy,the tumor volume reduced obviously,and the patients could be cured completely by further combination of chemotherapy with radiotherapy.Conclusion The diagnosis of basal ganglia germinomas should be considered in the male children around 11 years old with progressive hemiparesis,intelligent deficiency,high HCG level of serum or CSF,and the basal ganglia with no obvious boundary spaceoccupying lesion and the ipsilateral temporal lobe atrophy in neuroimaging.It can be further confirmed by diagnostic radiotherapy and then the patients should receive further chemotherapy with combination of radiotherapy so as to reduce the risk of surgery.