| Obejectives:Hepatic epithelial hemangioendothelioma(HEHE)is a rare tumor,with low incidence and unpredictable prognosis.At present,it is mostly reported in scattered cases.There is no universally accepted treatment strategy,the factors that affect the survival prognosis of patients are not clear,and the choice of treatment methods is lack of evidence.In this study,23 cases in our center were reported in detail,and 120 clinical data of patients screened from 1975 to 2016 in SEER database were analyzed to explore the etiology,diagnosis,treatment,clinical outcome and prognostic factors of this disease,so as to provide reference for the selection of clinical diagnosis and treatment strategies.Methods:This paper retrospectively analyzed the pathological data of 23 patients with hepatic epithelioid hemangioendothelioma diagnosed by pathology in Department of Hepatobiliary and Pancreatic Transplantation of the First Affiliated Hospital of Zhengzhou University from January 2012 to June 2020,and the clinical data of 23 patients with HEHE,and carries on the detailed report.The specific indicators include general population statistics data,clinical manifestations,imaging findings,laboratory examination and immunohistochemical markers of pathology,diagnosis,treatment and prognosis.All data were analyzed using SPSS21.0 statistical software.The measurement data were tested by t test.Kaplan.Meier method was used for survival analysis.P<0.05 indicates that the difference is statistically significant.We also collected clinical data on patients with pathologically diagnosed hepatic epithelioid hemangioendothelioma from 1975 to 2016 by the cancer statistics database SEER(Surveillance,Epidemiology,and End Results).It mainly included gender,race,age at diagnosis,marital status,tumor size,distant metastasis,surgical information,lymph node resection and follow-up data(survival time and survival status).Descriptive statistics were used to analyze patients’ baseline characteristics,and univariate and multivariate Cox regression models were used to evaluate the association between clinical variables and Overall survival(OS).Kaplan-Meier survival curve was used to compare the effects of various surgical treatments on survival of HEHE patients.SPSS software was used for statistical analysis.Results:1.Clinical data analysisTwenty-three patients were included,including 6 males and 17 females,with a male-to-female ratio of 1:2.83.The onset age of the patients ranged from 21 to 71 years old,with an average age of(44.35±13.43)years.The clinical manifestations were varied:6 cases were diagnosed with epigastric pain or discomfort,and 17 cases were incidentally found during physical examination.Imaging findings:85%(17/20)were hypoechoic in B-mode ultrasound examination;90%(18/20)plain CT scan showed low density.MRI examination showed low signal on T1WI and high signal on T2WI in all 7 patients.PET/CT showed that the radiation distribution of liver mass was concentrated in 8 cases(8/9,88.9%).Immunohistochemical results:endothelial cell differentiation marker CD34(23/23),CD31(23/23),F-Ⅷ(7/8),the FLI-1(12/12),ERG(17/17)were positive expression.Treatment methods and outcomes:12 of 23 patients received conservative treatment,and the 1,3,and 5-year survival rates were 82%,57.8%and 57.8%,respectively.The1,3,and 5-year survival rates of 11 patients who underwent surgical treatment were 100%,100%and 75%,respectively.2.SEER database analysisAmong the 120 HEHE patients,the most common treatment was non-surgical treatment(76/120,63.3%),followed by hepatectomy(20/120,16.7%)and liver transplantation(12/120,10.0%),and unknown treatment(12/120,10.0%).Survival rates were significantly higher in patients treated with surgery than in those treated with non-surgery(P=0.017),and there was no statistically significant difference in survival rates between patients treated with hepatectomy and liver transplantation(P=0.627).Univariate analysis showed that age at diagnosis(hazard ratio:2.28,P=0.003),surgical treatment(hazard ratio:2.68,P=0.037),and tumor size(hazard ratio:5.38,p<0.001)is a potential risk factor for survival in HEHE patients.Multivariate Cox hazard ratio regression analysis showed that surgical treatment(hazard ratio:16.04,P=0.012)and tumor size(hazard ratio:6.91,P<0.001)were independent prognostic factors affecting OS.Surgical treatment can reduce the risk of death and prolong OS,which are independent protective factors.Results of survival analysis also showed that surgical treatment was significantly more effective than non-surgical treatment,but there was no statistically significant difference between liver transplantation and hepatectomy(P=0.627).Conclusions:1.Hepatic epithelioid hemangioendothelioma,which is more common in middle-aged women,has no specific clinical symptoms and signs,no typical laboratory examination signs,and lacks specific tumor markers.Ultrasound examination has screening significance for early diagnosis of HEHE.The characteristic features of enhanced CT and enhanced MRI examination are "capsule retraction sign","target sign","lollipop sign" and fusion trend.The abnormal concentration of radioactivity distribution in 18F-FDG PET/CT examination has important auxiliary diagnostic significance.Histopathological immunohistochemical examination are required to confirm the diagnosis of HEHE,with the detection of endothelial marker CD31,CD34,and the FLI-1 and F-Ⅷ antigen.2.HEHE is a rare disease with low malignancy and slow progression.Surgical treatment should be performed actively in early detection,and liver transplantation should be actively considered even if the opportunity of surgical treatment is lost at the first diagnosis.Surgery and tumor size are closely related to the prognosis of patients.Surgical treatment can reduce the risk of death and improve the prognosis of patients,which is an independent protective factor.Surgical treatment can significantly improve the survival rate and prolong the survival time of patients.Liver transplantation and hepatectomy can achieve the same clinical effect. |
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