上皮样胶质母细胞瘤10例报告并文献复习

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...

Ten cases of epithelioid glioblastoma and review of literature

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.