CAJ | 학술논문

目的探讨肝脏上皮样血管内皮瘤(EHE)的临床和病理学特点,提高对该病的认识和诊治水平.方法回顾性分析2007年1月至2013年1月诊治的17例肝脏EHE患者的临床、放射学、病理学特征及治疗方法与患者生存情况.结果 17例肝脏EHE患者的平均年龄为46.2岁,其中女11例,男6例.7例患者有右上腹或肝区隐痛,其余10例无症状.多数患者(15例)肝功能正常.入院诊断多误诊为肝癌(转移性肝癌6例,原发性肝癌4例),3例患者术前发现已有肝外转移.肿瘤病灶以多发结节为主(10例),多见于包膜下.影像学检查可见包膜回缩征.病理学表现为小的细胞内管腔或空泡形成,内可见单个或数个红细胞.单个肿瘤细胞核偏位,类似印戒细胞.免疫组织化学以CD34和波形蛋白阳性率(分别为15/17和4/5)较高.手术切除(14例)为肝脏EHE的主要治疗措施,14例患者术后随访2至72个月仍健在.结论肝脏EHE发病率低,好发于中年女性,大多数患者无明显临床症状,实验室检查亦无明显异常,国内主要采用手术切除治疗,预后较好.
목적 탐토간장상피양혈관내피류(EHE)적림상화병이학특점,제고대해병적인식화진치수평.방법 회고성분석2007년1월지2013년1월진치적17례간장EHE환자적림상、방사학、병이학특정급치료방법여환자생존정황.결과 17례간장EHE환자적평균년령위46.2세,기중녀11례,남6례.7례환자유우상복혹간구은통,기여10례무증상.다수환자(15례)간공능정상.입원진단다오진위간암(전이성간암6례,원발성간암4례),3례환자술전발현이유간외전이.종류병조이다발결절위주(10례),다견우포막하.영상학검사가견포막회축정.병이학표현위소적세포내관강혹공포형성,내가견단개혹수개홍세포.단개종류세포핵편위,유사인계세포.면역조직화학이CD34화파형단백양성솔(분별위15/17화4/5)교고.수술절제(14례)위간장EHE적주요치료조시,14례환자술후수방2지72개월잉건재.결론 간장EHE발병솔저,호발우중년녀성,대다수환자무명현림상증상,실험실검사역무명현이상,국내주요채용수술절제치료,예후교호.
Objective To investigate the clinical and pathological features of primary hepaticepithelioid hemangio endothelioma (EHE) and try to improve the understanding and diagnosis of this disease.Methods From January 2007 to January 2013,the data of 17 patients with hepatic EHE were retrospectively analyzed,which included clinical,pathological and radiological characteristics,treatment and survival condition of these patients.Results Among 17 cases of hepatic EHE,there were 11 female and six male with the mean age of 46.2 years.Seven patients had right upper quadrant pain of dull pain in liver and the left 10 cases were without any symptoms.The liver function of most patients (15 cases) was normal.The admission diagnosis was mostly misdiagnosed as hepatocellular carcinoma (metastatic liver carcinoma six cases,primary liver carcinoma four cases).Extrahepatic metastasis was found in three patients before operation.The major lesion of the tumor was multiple nodules (10 cases) and most of which were under liver capsule.Retraction of the liver capsule was found in the imaging examination.The features of pathology were intracellular lumen and vacuoles formed in small cells,in which single or couple red blood cells could be found.Nuclear deviation was found in single tumor cell which looked like signet ring cells.The results of immunohistochemistry indicated the positive rate of factor vimentin (4/5) antigen and CD34 (15/17) was high.Surgical resection (14 cases) was the main treatment of hepatic EHE.Fourteen patients were still alive followed up for two to 72 months after operation.Conclusions The incidence of hepatic EHE is low,and it was susceptible to middle aged women and without obvious symptom.No obvious abnormality is found in laboratory examination.Surgical resection is the main treatment which enabled a good prognosis.