肝脏上皮样血管内皮瘤44例临床病理观察

Clinicopathological observation of hepatic epithelioid hemangioendothelioma:a study of 44 cases

目的 探讨肝脏上皮样血管内皮瘤(hepatic epithelioid hemangioendotheliom, HEHE)的临床病理特征。方法 收集2014年9月~2021年7月复旦大学附属中山医院诊断的44例HEHE,分析其临床特征、影像学、组织学特征、免疫表型、分子改变、治疗及预后,并复习相关文献。结果 44例中穿刺活检标本28例,手术切除标本16例。其中男性19例,女性25例,男女比为1∶1.3,年龄26~77岁,中位年龄51岁,平均50.5岁。临床多无症状,66%(29/44)为体检发现肝占位,34%(15/44)出现腹痛、上腹不适、下肢水肿等症状。单结节型10例(23%),多结节型34例(77%)。肿瘤细胞圆形、卵圆形、梭形或胖梭形,细胞轻~中度异型,核仁明显,部分呈印戒样,胞质空泡状,单个散在、巢片状或条索状排列,可见单细胞血管腔形成,腔内见红细胞。免疫表型:肿瘤细胞至少表达CD31、CD34、FⅧRAg、FLI-1、ERG中两种以上血管源性标记,Ki-67增殖指数为3%~30%。28例获得随访,其中7例无瘤生存,大部分带瘤生存或治疗后复发。结论 HEHE少见,虽为低度恶性血管内皮源性肿瘤,但其预后不确定,极易复发,诊断困难,确诊需结合组织学、免疫表型及分子检测。

Purpose To investigate the clinical features,histological morphology,immunohistochemistry,diagnosis,differential diagnosis and prognosis of hepatic epithelioid hemangioendothelioma(HEHE).Methods 44 cases of HEHE were collected from September 2014 to July 2021.The clinical features,imaging,histological morphology,immunohistochemical characteristics,molecular changes,treatment and prognosis were analyzed,and relevant literatures were reviewed.Results Among the 44 patients,28 cases were biopsy specimens and 16 were resection specimens.There were 19 male patients and 25 female patients,with a male to female ratio of 1∶1.3.The patients were 26-77 years old,whit a average age of 50.5 years.Most of them were asymptomatic clinically.About 66%of them were found to have liver occupying(29/44),and some of them had abdominal pain,upper abdominal discomfort,lower limb edema and other symptoms 34%(15/44).There were 10 cases(23%)of mononodular type and 34 cases(77%)of polynodular type.Histologically,the tumor cells were round,oval,epithelioid,and fat spindle,the tumor cells were mild to moderate dysplasia with prominent nucleoli.Some tumor cells were signet-ring like with cytoplasmic vacuoles,which arranged into single scattered,nested or cord.Formation of single-cell vascular lumen with red blood cells in lumen were seen.Immunohistochemistry,the tumor cells were positive for at least two vascular markers including CD31,CD34,FⅧRAg,FLI-1 and ERG.Ki-67 index were ranged from 3%to 30%.28 patients were followed up,most of them survived with tumor or recurred after treatment.Conclusion HEHE is a rare tumor.Although it is a low-grade malignant tumor of vascular endothelial origin,its prognosis is uncertain and prone to recurrence,and diagnosis is difficult.Diagnosis requires a combination of histological morphology,immunohistochemistry and molecular detection.