Objective To explore the clinicopathological feature,immunohistochemical and differential diagnosis of primary hepatic epithelioid hemangioendothelioma（ HEHE）. Methods The pathological features were investigated in seven cases of HEHE by HE and immunohistochemical staining,with review the correlative literatures. Results The gender of the patients were three females and four males. The symptoms were right upper quadrant abdominal pain. Only two cases showed hypohepatia. Five cases showed multiple liver lesions in computer tomography or MRI. Histologically,the epithelioid tumor cells which contained vacuoles and red blood cells in the cytoplasm were distributed scatteredly or in cords,accompanied by myxoid or fibrous stroma. Immunohistochemically,the tumor cells were diffusely positive for ERG,CD31,CD34 and F8; CD10 staining was positive in the majority cases. Some cases showed AE1 / AE3,SMA,and CK8 /18 positive staining. Three patients died in 1- 3 months after diagnosis,3 patients were alive with disease and 1 was lost in follow-up. Conclusions HEHE is a rare malignant vascular tumor with no significant specificity in clinical manifestation and examination. Its diagnosis needs to combine with histology and immunohistochemistry and rule out other differential diagnosis,when necessary,with FISH or RT-PCR to detect WWTR1-CAMTA1 fusion gene.
Chinese Journal of Diagnostic Pathology
Hepatic epitheliod hemangioendotheliama