Epithelioid hemangioendothelioma of the liver (HEH) was first reported by Ishak [and is more prevalent in middle-aged women, and a study by Arianeb Mehrabi et al. of 402 patients showed that it occurred in a 2:3 ratio of men to women with a mean age of 41.7 years.The pathogenesis of HEH The pathogenesis of HEH is still not fully understood, and may be related to oral contraceptives, progesterone levels, vinyl chloride pollution, viral hepatitis, and other factors, and has also been reported to be complicated by tumors such as gastric cancer and hepatocellular carcinoma. The clinical course of HEH is between hemangioma and hemangioendothelial sarcoma, and the clinical manifestations are atypical. 25% of patients are asymptomatic, and the remaining manifestations are mainly non-specific epigastric pain, hepatomegaly, malaise, ascites, jaundice, and also Budd-Chiari syndrome-like manifestations, and systemic metastasis and liver failure may occur in advanced stages. Laboratory tests may include high AKP and r-GT, and most of the tumor indicators are normal. Ultrasound of abdomen shows nodular or diffuse echogenic areas, mostly hypoechoic lesions, or hyperechoic or isoechoic clusters with peripheral hypoechoic border corona, and there seems to be no inevitable connection between the size of tumor and the height of the echo, also very few patients can show normal ultrasound. The CT scan of the abdomen can be divided into multinodular type and solitary type. multinodular nodules are common, with a diameter of 1-3 cm, and it has also been suggested that the solitary large nodular shadow is formed by the fusion of multinodular nodules. the CT manifestations are mostly hypodense shadow, with a small number of high-density shadow and heterogeneous mixed lesions. the rest of the manifestations include calcification, cystic constriction, compensatory hypertrophy in the uninvolved areas of the liver, splenomegaly, etc. Arianeb Mehrabi et al. summarized the imaging features of HEH: tumors are mostly located in the periphery of the liver, and sometimes extension or retraction of the hepatic envelope is seen. The tumor is mostly located in the peripheral part of the liver, and the pericardium is not dilated or wrinkled due to fibrosis. The tumor parenchyma enters the contrast agent after delay, and the central low-density area is not enhanced. In addition, compensatory hypertrophy of non-damaged liver tissues, portal hypertension, splenomegaly, and local calcification are also common manifestations of their abdominal CT. MRI shows low signal on T1-weighted image and high signal on T2-weighted image of the tumor. While hemorrhage, coagulative necrosis and calcification showed low signal in the central zone, edema of connective tissue and tumor hyperplasia showed high signal in the peripheral zone. Hepatic arteriogram showed abundant tumor vascularity and “bowl-like” staining of the subperitoneal lesions. The diagnosis of HEH relies on pathological examination. ehe is predominantly multinodular, with tumor tissue centered on oligocytic fibrosclerotic areas with mucinous or hyaline changes, and peripheral vascular rich areas, both distributed interdependently. The tumor cells are epithelial-like endothelial cells, round or polygonal in shape, large in size, with varying amounts of cytoplasm, pink and eosinophilic, with vacuolated nuclei and eccentric nuclei with small nucleoli. Some tumor cells are dendritic, stellate or spindle-shaped, with lightly eosinophilic cytoplasm and several finger-like protrusions. In addition, the tumor cells often infiltrate into the hepatic sinusoids and terminal hepatic veins, forming tumor plugs, which can become fibrotic or glassy and cause vascular occlusion. As the disease progresses, extensive calcification, necrosis, hemorrhage, and inflammatory reaction and infiltration of inflammatory cells are seen in the tumor interstitium. The tumor cells are composed of epithelioid or dendritic cells with intracytoplasmic vascular lumen formation, similar to indolent cell carcinoma. Immunohistochemistry was highly positive for vascular endothelial markers with factor VIII-related antigen (in almost all patients), CD34 (94%), and CD31 (86%). There are also some EHE with atypical pathological presentation that can be easily confused with sclerosing hemangioma and adenocarcinoma such as cholangiocarcinoma. Positive intracytoplasmic mononuclear cell vascular lumen and vascular endothelial markers are the two characteristic manifestations of HEH. Therefore, histology and immunohistochemistry play a decisive role in the diagnostic process, so high requirements are needed for both the biopsy specimen and the pathologist. In this case, the diagnosis was not clearly made by liver puncture and was only clearly diagnosed by pathology and immunohistochemistry after surgical resection. Due to the non-specific imaging presentation of EHE, it is often misdiagnosed as metastatic tumor. The CT features of metastatic tumor are hypointense shadow, which also shows peripheral enhancement and “bull’s eye sign” after enhancement, similar to EHE. The key point of differentiation is that pathological examination shows little heterogeneity of EHE, rare nuclear schizophrenia, and immunohistochemistry expresses endothelial cell markers, while metastatic expresses epithelial markers of the primary tumor. In addition, due to the presence of residual and proliferating small bile ducts in EHE, it is often indistinguishable from cholangiocarcinoma. The main points of differentiation between the two, in addition to cellular heterogeneity, are that EHE expresses endothelial markers of vascular endothelium such as CD34, CD31, Vimentin, and factor VIII positively, while CK18 and CK19 associated with cholangiocarcinoma are negative. Epithelioid hemangioendothelioma of the liver is insensitive to chemotherapy and radiotherapy. Some studies have shown that hepatic artery embolization chemotherapy (Transarterial chemoembolization, TACE) can be used for patients waiting for liver transplantation, and the drugs for embolization chemotherapy can be chosen from mitomycin, 5-fluorouracil, adriamycin, etc. Some patients had tumor shrinkage after TACE, but due to sample size is very small, further studies are still needed. In a study by Arianeb Mehrabi et al. on 128 patients who underwent liver transplantation, the 1-year and 5-year survival rates were 96% and 54.5%, respectively. For patients who cannot or are not eligible for liver transplantation, surgical treatment is the treatment of choice. Among the pharmacological treatments, thalidomide is worth mentioning, which can be tried in diffuse metastatic HEH because of its vascular endothelial inhibitory effect. Makhlouf et al. concluded that the density of tumor cells and tumor cell necrosis correlate with clinical prognosis, and there are also data showing that combined hepatocellular liver cancer and tumor diameter >7 cm have poor prognosis.