基底节区生殖细胞瘤的诊断及鉴别诊断

Diagnosis and Differential Diagnosis of Germ Cell Tumors in the Basal Ganglia

目的总结并分析基底节区生殖细胞瘤的临床及影像学特征,提高对该病的早期诊断水平。方法收集2013年1月至2023年9月北京协和医院收治的基底节区生殖细胞瘤患者的临床及影像资料,对其进行回顾性分析。结果18例基底节区生殖细胞瘤患者(男16,女2)多表现为偏侧肢体无力。所有患者AFP均正常,7例(7/18)血清β-HCG升高,8例(8/15)脑脊液β-HCG升高。共发现19个病灶(1例双侧),MRI表现为Ⅰ型的有7例,T2WI呈斑片状稍高信号,无强化;7例为Ⅱ型,表现为斑片状、类圆形T1WI低信号,T2WI高信号,结节状强化(病灶<3cm);5例为Ⅳ型,表现为囊实性团块影,显著强化(病灶≥3cm)。14例(14/18)出现同侧大脑脚萎缩,8例(8/18)出现同侧大脑半球萎缩。结论基底节区生殖细胞瘤好发于10-14岁男性,影像分型不同但各具特点,结合偏侧肢体无力的临床表现及血清和脑脊液肿瘤标志物(AFP、β-HCG)检查可早期诊断。

Objective Summarize and analyze the clinical and imaging features of germ cell tumors in the basal ganglia(BGG).Methods Retrospective analysis of clinical and imaging data of BGG patients admitted to Beijing Union Medical College Hospital from January 2013 to September 2023.Results Most of the 18 patients with BGG showed unilateral limb weakness.All patients had normal AFP,and 7 cases(7/18)serumβ-HCG elevated,8 cases(8/15)cerebrospinal fluidβ-HCG elevation.A total of 19 lesions were found(1 case bilateral),MRI findings:Type I(7 patients)showing patchy and slightly high signal intensity on T2WI,without enhancement;Type II(7 patients)manifested as patchy and round T1WI low signal,T2WI high signal,nodular enhancement(lesion<3cm);Type IV(5 patients)manifestated as cystic solid mass shadow with significant enhancement(lesion≥3cm).14 cases(14/18)showed ipsilateral cerebral foot atrophy,8 cases(8/18)showed ipsilateral cerebral hemisphere atrophy.Conclusion BGG commonly seen in males aged 10-14,different imaging subtypes have their own characteristics,combined with clinical manifestations of hemiplegia and serum and cerebrospinal fluid tumor markers(AFP、β-HCG)examination can make early diagnosis.