肝脏上皮样血管内皮瘤穿刺标本的病理诊断与鉴别诊断

Hepatic epithelioid hemangioendothelioma in needle biopsy specimens： report of 5 cases with review of literature

目的 提高对经皮肝穿刺活检标本中上皮样血管内皮瘤（EH）的认识.方法 收集北京肿瘤医院1999-2010年3016例肝穿刺活检标本中5例诊断EH的病例,回顾性分析5个病例的光镜下病理形态学特点、免疫组织化学（polymer二步法）检测结果,以及患者的临床表现、实验室检查和影像学结果.结果 5例均为女性,年龄23～47岁,平均39岁.4例B超显示肝多发实性肿物并考虑转移癌;实验室检查各项指标未见明显异常.光镜下肿瘤细胞上皮样、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,可见明显的胞质内空泡;瘤细胞形态温和,未见核分裂;免疫组织化学表达血管标志物CID31、CD34、第八因子相关抗原,偶见表达平滑肌肌动蛋白.结论肝穿刺活检标本中,如果病变具有典型的EH形态特点和免疫组织化学表达,同时又有临床及影像学支持,即可明确诊断;应用多种抗体组合有助于肝穿刺活检标本EH的诊断与鉴别诊断.

Objective To evaluate the pathologic diagnosis of hepatic epithelioid hemangioendothelioma （EH） in needle biopsy specimens. Methods Five cases of hepatic EH diagnosed in needle biopsies encountered during the period from 1999 to 2010 in Beijing Cancer Hospital were retrospectively reviewed. The specimens were formalin-fixed, paraffin-embedded and stained with hematoxylin and eosin. Immunohistochemical study was also carried out. Results All the 5 patients were females. The age ranged from 23 to 47 years （ mean = 39 years）. The tumors in 4 patients were multiple and diagnosed as ＂metastasis＂ on ultrasound examination. The blood test results in all of the 5 patients were normal. Histologically, the tumor cells had an epithelioid appearance and were arranged in cords, solid nests or isolation, amongst a myxoid or hyaline matrix. The tumor cells contained scattered intracytoplasmic vacuoles which sometimes harbored red blood cells. There was no evidence of significant cellular pleomorphism, high mitotic activity and necrosis. Immunohistochemically, all of the 5 cases were positive for at least two endothelial markers （CD31, CD34 and factor Ⅷ-related antigen）. Smooth muscle actin was expressed in 1 case. Conclusions The diagnosis of hepatic EH can be established in needle biopsy specimens. The histologic pattern, when coupled with immunohistochemical findings, is useful in arriving at the correct diagnosis.