CAJ | 학술논문

目的探讨肝上皮样血管内皮瘤(hepatic epithelioid hemangioendothelioma,HEHE)的临床特点、诊断与鉴别诊断要点.方法对6例经肝穿刺诊断为HEHE患者的临床资料和病理学特点进行分析.结果男2例,女4例,年龄25~58岁,平均年龄45岁.临床主要表现为右上腹疼痛、发热、乏力、消瘦.2例无任何症状.病理及影像学表现为5例肝内多发,1例单发,3例有肺部转移.组织学特征:呈短梭形具有血管分化的树突状细胞和有胞质内血管腔的上皮样细胞,间质致密纤维化及黏液样变.免疫组化:6例CD34全部阳性,5例CD31阳性,5例Vimentin阳性,CK、FⅧRAg偶为阳性.3例接受了肝脏介入栓塞治疗,其中1例还接受了氩氦刀冷冻消融治疗及化疗;1例给予肿瘤微波消融治疗;1例等待肝移植;1例未治疗.随访8月~14年,5例健在,1例失访.结论 HEHE为较为罕见的肿瘤,确诊依靠病理及免疫组织化学诊断,鉴别诊断需排除转移性腺癌及胆管癌;治疗方式为手术或介入治疗.
목적탐토간상피양혈관내피류(hepatic epithelioid hemangioendothelioma,HEHE)적림상특점、진단여감별진단요점.방법대6례경간천자진단위HEHE환자적림상자료화병이학특점진행분석.결과남2례,녀4례,년령25~58세,평균년령45세.림상주요표현위우상복동통、발열、핍력、소수.2례무임하증상.병리급영상학표현위5례간내다발,1례단발,3례유폐부전이.조직학특정:정단사형구유혈관분화적수돌상세포화유포질내혈관강적상피양세포,간질치밀섬유화급점액양변.면역조화:6례CD34전부양성,5례CD31양성,5례Vimentin양성,CK、FⅧRAg우위양성.3례접수료간장개입전새치료,기중1례환접수료아양도냉동소융치료급화료;1례급여종류미파소융치료;1례등대간이식;1례미치료.수방8월~14년,5례건재,1례실방.결론 HEHE위교위한견적종류,학진의고병리급면역조직화학진단,감별진단수배제전이성선암급담관암;치료방식위수술혹개입치료.
Objective To study the clinical characteristics, diagnosis and differential diagnosis of hepatic epithelioid hemangioendothelioma(HEHE). Methods Clinical data and pathological characteristics of 6 patients with HEHE diagnosed by hepatic puncture were analyzed. Results Of the 6 HEHE patients, 2 were males and 4 were females at a mean age of 45 years (range:25~58 years). Their main clinical manifestations were right upper abdominal pain, fever, fatigue, and weight loss. Two patients were asymptomatic. Pathology and imaging showed multiple intra-hepatic metastases in 5 patients, single metastasis in 1 patient, and lung metastasis in 3 patients. Histology revealed short spindle cells characterized by dendritic and epithelioid cells, epithelial cells in cytoplasm, interstitial compact fibrosis and mucoid degeneration. Immunohistochemistry demonstrated positive CD34 in 6 patients, positive CD31 in 5 patients, positive vimentin in 5 patients, occasional positive CK and FVIIIRAg. Of the 3 patients who received hepatic embolism intervention, 1 underwent additional cold ablation and chemotherapy, 1 received additional microwave ablation therapy, 1 was going to undergo liver transplantation. The patients were followed up for 8 months~14 years, during which 5 were alive and 1 missed follow-up. Conclusion HEHE is a rare tumor. Its final diagnosis depends on pathological and immunohistochemical findings. It should be differentially diagnosed from metastatic adenocarcinoma and cholangiocarcinoma, and treated by surgical operation or intervention.