Three Cases Report Of Hepatic Epithelioid Angiomyolipoma And Review Of The Article

Objective:To provide the basis for accurate diagnosis and treatment of hepatic epithelioid angiomyolipoma(HEAML)we analysis 3 cases of hepatic epithelioid angiomyolipoma cases and review the relevant reports of hepatic epithelioid angiomyolipoma in recent years,summarized the clinical characteristics and treatment methods?Methods:Retrospective analysis three cases who were diagnosed as hepatic epithelioid angiomyolipoma,according to the postoperative pathology and immunohistochemical verificatio in the hepatobiliary pancreatic surgery,Second Affiliated Hospital of Da Lian Medical University from December 2015 to December 2016.The general state,clinical symptoms,imaging manifestations,histological results and immunohistochemistry of these patients were retrospectively reviewed and analyzed.Studies of HEAML published from January 2000 to December 2016 were collected from Pub Med to summarize the clinical symptoms,imaging characteristics,treatment,outcome and research status of HEAML.Results:All the three cases were female patients.The mean age is 53±2.8 years(ranging from 49-55years).They all suffered from the single solid tumor of which the average size was 2.03±0.82cm(ranging from 1 to 3cm).One patient went to the hospital due to abdominal discomfort,and the other 2 patients were accidentally found with tumors during the routine physical examination without obvious discomfort.The routine blood tests,biochemical examinations of liver,detection of virus antigen and antibody for HBV and HCV,tumor-associated antigen tests were conducted at admission,and the results were all normal except that one patient had HBs AB(+),HBe AB(+),HBc AB(+),total bilirubin of 26?mol/L and conjugated bilirubin of 19?mol/L.The enhanced MRI indicated the low T1 signal intensity,high T2 signal intensity,significantly enhanced arterial phase,and slightly fading portal venous phase and lag phase.One case patient was given the Gd-EOB-DTPA-enhanced MRI and the slightly low T1 WI signal intensity and slightly high T2 WI signal intensity as well as significantly enhanced DWI lesion signal intensity.During dynamic enhancement,there were uneven and obvious enhancement over the whole lesion for the arterial phase,the obvious fading in the lesion for the portal venous phase,the low signal in the body of the lesion for the hepatocellular phase,and the equisignal in the lesion peripheries.All of the three patients were diagnosed as the primary hepatocellular carcinoma during the preoperative imaging examinations.One patient had the complication of other microlesions,and underwent the fine needle aspiration biopsy before the operation,the result of which was diagnosed as HEML in the larger tumor as evidenced by the immunohistochemistry.All of the 3 patients havereceived the surgical treatment.Postoperative pathologic observation showed that,the tumors were gray-yellow and gray-red in color and there were clear interfaces between the tumors and the normal hepatic tissues in 2 cases,but not in one case.The light microscope observation of the tumor tissues showed that one case had partial capsule,one case had infiltrated hepatic tissues accompanied with cellular atypia and clear or oncocytic cytoplasm.The pathological structure study showed that the tumors were consisted of the epithelioid cells with disordered arrangement and the adipocytes and thick-walled vessels in different proportions.The contents of the epithelial cells were above 60%.The immunohistochemical results showed the biphase expression(S-100(-)both for the melanocyte markers(HMB45 and Melan-A)and for the smooth muscle cell markers(SMA)in the 3 cases.After retrieving in PUBMED,29 eligible papers were found,containing 84 cases mostly in the form of case reports.More than half of the patients(39/68,57.4%)were asymptomatic and accidentally found with tumor during physical examination or imaging examination.About 6.4%(5/78)patients had the complication of tuberous sclerosis.About 87.2%(48/55)patients were misdiagnosed with the hepatic carcinoma or other benign tumors before the operation.About 12%(9/75)patients had postoperative recurrence or metastasis(3 cases of distant metastasis),and this risk is high enough to be worthy of caution.Conclusion:Currently only rely on imaging examination to diagnosis hepatic epithelioid angiomyolipomas(HEAML)is difficult,preoperative diagnosis should be combined with biopsy pathologic examination.In patients who confirmed as HEAML preferred surgical treatment,its prognosis is good,the postoperative should regularly review.