目的 探讨原发性肝脏黏液表皮样癌(primary mucoepidermoid carcinoma of the liver,PMCL)的临床病理特征.方法 回顾性分析4例PMCL患者的病史、临床资料、病理诊断、鉴别诊断、治疗及预后等.结果 4例肿瘤均位于左半肝,单发;镜下瘤细胞由三种细胞构成:表皮样细胞、中间细胞、黏液细胞,所占比例各有不同.免疫表型:肿瘤细胞CA199、CK19弥漫强阳性,黏液细胞CEA局灶阳性,CK7主要表达于表皮样细胞及黏液细胞,CK5/6、p40、p63表达于表皮样细胞及中间细胞;特殊染色AB、PAS、黏液卡红染色证实黏液细胞存在,Ki67增殖指数30％～40％.FISH检测:4例PMCL均未检测到MAML2基因断裂重排,1例肿瘤发生MAML2基因扩增.结论 PMCL发病率低,临床表现无特异性,极易误诊,诊断时需与胆管腺癌、腺鳞癌等相鉴别,且无明确的标准治疗方案,预后差.
Primary hepatic mucoepidermoid carcinoma:clinicopathologic analysis of 4 cases and literature review
Purpose To investigate the clinicopathological features of primary mucoepidermoid carcinoma of the liver(PMCL).Methods The history,clinical data,pathological diagnosis,differential diagnosis,treatment and prognosis of 4 patients with PMCL were retrospectively analyzed.Results All four tumors were located in the left half of the liver and were sol-itary.Microscopic examination showed that tumor cells were composed of three types of cells:epidermal like cells,interme-diate cells,and mucinous cells,with varying proportions.Im-munophenotype:tumor cells showed diffuse strong positivity for CA199 and CK19,while mucous cells showed focal positivity for CEA.CK7 mainly expressed in epidermal like cells and mucous cells,while CK5/6,p40,and p63 expressed in epidermal like cells and intermediate cells.Special staining with AB,PAS,and mucin carmine confirmed the presence of mucous cells,Ki67 proliferation index ranged from 30％to 40％.FISH testing:no MAML2 gene breakage or rearrangement was detected in 4 cases of PMCL,and MAML2 gene amplification occurred in 1 case of tumor.Conclusion The incidence of PMCL is low and present with non-specific clinical manifestations,which often leads to misdiagnosis.The diagnosis should be differentiated from bile duct adenocarcinoma and adenosquamous carcinoma.Moreover,there is currently no established standard treatment for PMCL and has a poor prognosis.
万方数据
维普
