肝上皮样血管内皮瘤临床及组织学观察  

Hepatic epithelioid haemangioendothelioma:A clinicpathologic analysis

作　　者：崔华娟[1] 廖秋林[1] 王炜[1] 王卓才[1] 彭大云[1] 赖续文[1] 

机构地区：[1]广州军区广州总医院病理科,广东广州510010

出　　处：《中华肿瘤防治杂志》2013年第6期458-461,共4页Chinese Journal of Cancer Prevention and Treatment

摘　　要：目的:分析肝脏原发的上皮样血管内皮细胞瘤(EHE)临床与病理表现,以提高对EHE的认识。方法:回顾性分析4例EHE的临床、影像、组织学形态及免疫组化表达及特殊染色结果,结合随访结果复习相关文献进行讨论。结果:4例患者中,男2例,女2例,年龄分别为55、36、41和44岁。临床表现为腹胀、右上腹痛,其中1例非常罕见的类似布加氏综合征(BCS)的表现。2例肝功能异常。影像学上均表现为肝内多发结节或弥漫性病变,临床考虑肝癌或BCS。3例为肝脏穿刺标本,1例为肝脏全切标本,后者切面见弥漫性灰白色病变,与周围肝组织分界不清。光镜下细胞形态以上皮样细胞为主,排列成短条索状或小巢状,包埋于特征性黏液玻璃样基质中。瘤细胞胞质丰富,可见明显的胞质内空泡似印戒样。3例穿刺标本细胞形态温和,未见核分裂相,2例有坏死。1例肝脏全切标本细胞异型性较明显,可见核分裂相,>1/10HPF,并见大片凝固性坏死。免疫组化示,CD31、CD34、F8及VEGF阳性,广谱CK偶见灶性阳性。特殊染色瘤细胞PAS及AB-PAS均阴性。随访2～22个月,1例肝移植术后死亡,2例出院后死亡,1例失访。结论:EHE为一种少见的恶性肿瘤,临床与影像学表现无特异性,极易误诊为其他肝脏肿瘤,需要引起重视。OBJECTIVE： To improve the knowledge of hepatic epithelioid haemangioendothelioma（EHE）. METH- ODS： To investigated and review the clinicpathologic features of four cases of EHE by light microscopic observation and immunohistochemical method and reviewed the correlative literatures. RESULTS： The gender of the patients were two fe- males and two males. The age were 55,36 for females and 41 and 44 years old for males respectively. The symptoms were right costal or abdominal pain,windy and one patient with symptoms resemble Budd-Chiari syndrome. Two cases showed hypohepatia. Imaging studies revealed multiple nodular or diffuse damage and misdiagnosed as ＂liver cancer＂ or ＂Budd- Chiari syndrome＂. Three cases were diagnosed by needle biopsy and one case underwent hepatectomy. Histologically, the tumor cells had a epithelioid appearance and were arranged in cords or solid nests, amongst a myxoid or hyaline matrix. The tumor cells had abundance cytoplasm and contained scattered intracytoplasmic vacuoles just like ring cells. The tumor cells of three needle biopsy specimens lacked pleomorphism,mitotic activity whereas the tumor cells of hepatectomy speci- mens had obvious pleomorphism and mitotic activity 〉1/10 HPF. Massive necrosis could been seen. They were immuno- histochemically positive for CD31 ,CD34 ,FⅧ and VEGF. CK staining was positive in some cases,PAS and AB-PAS stain were negative. The follow-up time was 2--22 months. One patient died after operation, 2 patient died after leaving hospi- tal, and 1 was lost in follow-up. CONCLUSIONS： Hepatic epithelioid hemangioendothelioma is malignancy and represents a distinct clinical pathological entity. It is rare and often misdiagnosed as other liver tumors.

关 键 词：肝脏 上皮样血管内皮细胞瘤 临床 病理分析 回顾性研究 

分 类 号：R735.7[医药卫生—肿瘤]