杨柳松, 祝迎锋, 唐剑敏. Ten cases of epithelioid glioblastoma and review of literature[J]. China Oncology, 2021, 31(2): 114-120. DOI: 10.19401/j.cnki.1007-3639.2021.02.005.
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