Hepatic Epithelioid Haemangioendothelioma: A Comprehensive Review Of The Literature

Objective:Hepatic epithelioid hemangioendothelioma(HEHE) is a rare malignant tumor of vascular origin with incidence below 0.1/100,000.We report two cases of HEHE.Then we present a comprehensive review of the literatures on HEHE to recognize its clinical features,diagnostic approaches,therapeutic modalities,and clinical outcome.Methods:We report two cases diagnosed by the 2nd affiliated hospital of Zhejiang University,College of Medicine.Then we using computer search the HEHE in the database of MEDLINE and Chinese Journal of Science and Technology of VIP.We present a comprehensive review of the literatures.The reviewed parameters include demographic data,clinical manifestations,diagnostic approaches,therapeutic modalities,and clinical outcome.Result:1.General information:All published series on patients with HEHE(n=125 paper, n=574 patients) are analyzed from the first description in 1984 to the current literature. The mean age of patients with HEHE is 40.9 years,and the male-to-female ratio is 2:3. The etiology of this disease is still unknow.Some possible risk factors are oral contraceptive pills,poly vinyl chloride,asbestos,thorotrast contact,hepatic trauma, and virus hepatitis.2.Clinical features:It has variable clinical manifestations.22.7%patients are asymptomatic,while 77.3%patients have clinical manifestations.The most common clinical manifestations are right upper quadrant pain(49.9%),hepatosplenomegaly (25.7%),distension or abdominal discomfort(16.0%),and weight loss(15.2%).Most patients presented with multifocal tumor that involve both lobes of the liver(81.9%), 13%patients are unifocal involved.The right lobe is affected more than the left lobe in both multifocal and unifocal.26.4%patients have extrahepatic involvement,The lungs (8.8%),regional lymph nodes(5.5%),peritoneum(4.7%),bone(3.3%),spleen(2.5%), and diaphragm(1.1%) are the most common sites of extrahepatic involvement at the time of diagnosis.78.9%patients have abnormal liver function,most common change are elevated AKP(66.9%),r-GT(41.9%),AST(27.9%),ALT(22.1%),Total Bilirubin (19.8%).Tumor markers(AFP,CEA,CA199) are always in the normal level.3.Diagnosis:Its diagnosis majorly depends on radiology and historic examination. Hypoechoic lesions on sonography(69.7%),hypodense lesions on CT scan(98%), peripheral enhancement on contrast-enhanced scans(81.5%),T1-weighted images reveal low intensity(90.3%),and heterogeneously increased signal on T2-weighed images(59.7%) are the most common results.The histopathological changes show marks of endothelial cell differentiation stained positive,such as factorâ…§-related antigen(97.2%),CD34(95%),CD31(86.8%),are much important in diagnosis.4.Therapeutic modalities:The most common management has been liver transplantation(LTx)(51.8%of patients),followed by no treatment(18.7%of patients), chemotherapy and radiotherapy(17.0%of patients),and liver resection(LRx)(12.5% of patients).The 1-year and 5-year patient survival rates are 96%and 54.5%, respectively,after LTx;39.3%and 4.5%,respectively,after no treatment,73.3%and 30%,respectively,after chemotherapy or radiotherapy;and 100%and 75%,respectively, after LRx.Conclusion:Hepatic epithelioid hemangioendothelioma is a rare tumor of vascular origin.It behaves as a low-moderate grade malignant tumor with the malignance between hemangioma and hemangioendothelioma.For its nonspecific manifestation,and variable imaging findings,it's hard to differentiate.Its diagnosis depends on historic examination.LRx has been the treatment of choice in patients with resectable HEHE. However,with other malignant liver neoplasm,LTx has been proposed as the treatment of choice because of the hepatic multicentricity of HEHE.In addition,LTx is an acceptable option for patients who have HEHE with extrahepatic manifestation.The role of different adjuvant therapies for patients with HEH remains to be determined.