临床儿科杂志 ›› 2020, Vol. 38 ›› Issue (11): 847-.doi: 10.3969/j.issn.1000-3606.2020.11.011

• 神经系统疾病专栏 • 上一篇    下一篇

基底节区生殖细胞瘤 7 例临床分析

姚春美 1, 邓亚仙 1, 王雅洁 1, 高丽梅 2, 高宝勤 1   

  1. 1 . 首都医科大学附属北京天坛医院儿科(北京 100070); 2 . 北京市大兴区人民医院(北京 102600)
  • 出版日期:2020-11-15 发布日期:2020-11-06
  • 通讯作者: 高宝勤 电子信箱:gaobaoqin@shohu.com

Clinical features of 7 patients with basal ganglia germinoma

YAO Chunmei 1 , DENG Yaxian1 , WANG Yajie 1 , GAO Limei 2 , GAO Baoqin1   

  1. 1 . Department of Pediatrics, Beijing Tiantan Hospital, Capital Medical University, Beijing 100070 , China; 2. Department of Stomatology, Daxing District People's Hospital, Beijing 102600, China
  • Online:2020-11-15 Published:2020-11-06

摘要: 目的 总结基底节区生殖细胞瘤(BGC)的临床特征。方法 回顾分析7例确诊BGC患儿的临床资料。结 果 7例患儿中男6例、女1例,中位年龄10岁8个月,自发病到确诊的中位时间为3个月。临床表现以偏侧肢体无力最常见, 随病程发展可出现构音障碍、认知功能减退等。所有患儿甲胎蛋白和β-人绒毛膜促性腺激素均无异常,部分患儿脑脊液特 异性寡克隆区带(SOB)阳性;影像学表现以单侧基底节萎缩伴大脑脚萎缩常见,也可累及双侧基底节。7例患儿均经立体定 向活检组织病理学检查确诊。结论 BGC以偏侧肢体无力常见,早期影像学表现不典型,以基底节伴大脑脚萎缩为主。

关键词: 生殖细胞瘤; 基底节; 甲胎蛋白; β-人绒毛膜促性腺激素

Abstract: Objective To summarize the clinical characteristics of patients with basal ganglia germinoma (BGC). Method The general information, clinical manifestations, laboratory examination and imaging results of seven patients with BGC were retrospectively analyzed. Results A total of seven patients diagnosed with BGC were retrospectively analyzed, including six males and one female, with a median age of ten years and eight months. The median time from onset to diagnosis was three months. The most common clinical manifestations were lateral limb weakness. Alpha-fetoprotein and beta-human chorionic gonadotrophin were normal, and specific oligoclonal bands in cerebrospinal fluid was positive in some patients. The common imaging findings were unilateral basal ganglia atrophy accompanied by cerebrum atrophy, and bilateral basal ganglia can also be involved. All the seven patients were confirmed by stereotactic biopsy histopathology. Conclusions BGC is characterized by lateral limb weakness and early imaging findings were atypical. The imaging features are the basal ganglia and cerebral peduncle.

Key words: germinoma; basal ganglia; alpha-fetoprotein; beta-human chorionic gonadotrophin