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