中国癌症杂志 ›› 2021, Vol. 31 ›› Issue (2): 114-120.doi: 10.19401/j.cnki.1007-3639.2021.02.005

• 论著 • 上一篇    下一篇

上皮样胶质母细胞瘤10例报告并文献复习

杨柳松 1 ,祝迎锋 2 ,唐剑敏 2   

  1. 1. 复旦大学附属华山医院北院神经外科,上海 201907 ;
    2. 复旦大学附属华山医院北院病理科,上海 201907
  • 出版日期:2021-02-28 发布日期:2021-03-02
  • 通信作者: 杨柳松 E-mail: billyong01@163.com

Ten cases of epithelioid glioblastoma and review of literature

YANG Liusong 1 , ZHU Yingfeng 2 , TANG Jianmin 2#br#   

  1. 1. Department of Neurosurgery, Huashan Hospital North, Fudan University, Shanghai 201907, China; 2. Department of Pathology, Huashan Hospital North, Fudan University, Shanghai 201907, China
  • Published:2021-02-28 Online:2021-03-02
  • Contact: YANG Liusong E-mail: billyong01@163.com

摘要: 背景与目的:上皮样胶质母细胞瘤(epithelioid glioblastoma,EGBM)是一种罕见的胶质母细胞瘤亚型,国内外报道较为少见。探讨EGBM的临床、病理学及影像学特征。方法:回顾性分析2014—2019年在华山医院北院经手术后病理学检查证实的10例EGBM的临床资料,对其临床、病理学及影像学特征进行归纳总结,并复习相关文献。结果:本组患者平均年龄42.2岁,平均病程60.3 d,常见症状、体征为头痛、头晕和局灶性神经功能障碍。影像学上肿瘤均位于幕上,伴不均匀强化和瘤周水肿,常见囊变。病理学上肿瘤细胞均有上皮样改变,部分有横纹肌样特征,胶质瘤的肿瘤标志物如胶质纤维酸性蛋白(glial fibrillary acidic protein,GFAP)、S100、P53多为阳性;可见上皮样标志物,如上皮膜抗原(epithelial membrane antigen,EMA);但异柠檬酸脱氢酶-1(isocitrate dehydrogenase-1,IDH-1)一般阴性。8例患者得到随访,但预后不一,有短期内复发并转移者,也有长期生存者,中位总生存时间(overall survival,OS)为17.0个月,中位无进展生存时间(progression-free survival,PFS)为14.0个月。结论:EGBM是一种少见的胶质母细胞瘤分型,具有一些特定的临床和影像学表现,但诊断主要依赖病理学和免疫组织化学检查,手术切除和术后放化疗仍是EGBM的主要治疗方法,EGBM患者的预后不一,总体虽差但仍有长期生存者,可能与其具有不同的分子生物学亚型有关;特有的分子生物学特征如BRAFV600E突变等对EGBM诊断和治疗的影响有待更多的研究证实。

关键词: 上皮样胶质母细胞瘤, 手术治疗, 影像学, 病理学, 预后

Abstract: Background and purpose: Epithelioid glioblastoma (EGBM) is a rare subtype of glioblastoma. This study aimed to learn more about the clinical, radiological and pathological features of EGBM. Methods: This study retrospectively analyzed 10 cases of EGBM verified by surgery at Huashan Hospital North from 2014 to 2019, including clinical manifestations, histological and radiological features and relevant literatures. Results: The mean age of patients with EGBM was 42.2 years, and the mean symptom duration was 60.3 days. Headache, dizziness and focal neurological dysfunction were the most common symptoms and signs. Supratentorial locations, heterogeneous enhancement and peritumoral brain edema were the common features of EGBM detected by magnetic resonance imaging (MRI), and concurrent with cystic change in about 40.0% patients. Histologically, all of EGBM had epithelioid components, and some of them also had rhabdoid components. Most of glioma biomarkers, such as glial fibrillary acidic protein (GFAP), S100 and P53, were positive in EGBM. Some of epithelioid biomarkers, such as epithelial membrane antigen (EMA), were also positive, while isocitrate dehydrogenase-1 (IDH-1) was negative. Eight cases were followed up, though they had different prognosis, EGBM in some patients recurred and metastasized quickly, while others had long overall survival (OS). Median OS and progression-free survival (PFS) were 17.0 and 14.0 months, respectively. Conclusion: EGBM is a rare type of glioblastoma with some distinct clinical and radiological features, however, its diagnosis depends on pathological and immunohistochemical findings. Surgery and postoperative chemoradiotherapy are nowadays still the main treatment methods of EGBM. The different prognosis of EGBM may be due to the different molecular biological subtypes. The molecular biological feature, such as BRAF V600E mutation, may influence the diagnosis and treatment of EGBM in the future, which needs further study.

Key words: Epithelioid glioblastoma, Surgery, Radiology, Pathology, Prognosis