肝的淋巴上皮瘤样癌5例临床病理分析

Lymphoepithelioma-like carcinoma in liver: a clinicopathological analysis of five cases

目的探讨肝的淋巴上皮瘤样癌(LELC)基本病理形态及临床预后特点。方法收集国家癌症中心病理科确诊的5例肝的淋巴上皮瘤样癌术后病例,回顾性分析其病理形态、免疫表型及临床预后特点,并复习相关文献。结果 5例中有2例是淋巴上皮瘤样型胆管细胞癌(LEL-CC),3例是淋巴上皮瘤样型肝细胞癌(LEL-HC);其中2例LEL-CC显示EBER原位杂交染色(+),而3例LEL-HC显示EBER(-);5例LELC血清AFP水平正常或轻度升高;3例合并乙型肝炎;1例合并肝硬化。2例LEL-CC随访10年及28个月,无病生存,而3例LEL-HC其中1例2年后死亡,1例2年后复发,另外1例随访1年无病生存。结论肝的LELC具有独特的临床病理特征,LEL-HC通常显示EBER(-),而LEL-CC常显示EBER(+)。与3例LEL-HC相比,2例LEL-CC显示预后相对较好。但因现有病例数过少,还需积累更多的病例进一步分析其临床预后特征。

Objective To investigate clinicopathological characteristics and outcomes of lymphoepithelioma-like carcinoma(LELC) in the liver.Methods Five postoperative specimens of LELC of the liver were diagnosed and retrieved from the Department of Pathology,National Cancer Center,and their morphology,immunophenotype and clinical outcomes were analyzed.The literatures were also reviewed.Results Among the 5 cases,2 were lymphoepithelioma-like cholangiocarcinoma(LEL-CC) and 3 were lymphoepithelioma-like hepatocellular carcinoma(LEL-HC).2 LEL-CCs showed positive EBER staining,whereas 3 LEL-HCs showed negative EBER staining.Serum AFP was normal or only slightly elevated in the 5 cases.Hepatitis B surface antigen was detected in 3 cases,and hepatic cirrhosis was found in 1 case.After 10 years and 28 months of follow-up respectively,no recurrence and death was found in the 2 LEL-CCs.Among the 3 cases of LEL-HCs,one patient died from disease after 2 years,one patient recurred after 2 years,and the other one was alive without disease after 1-year follow-up.Conclusions The LELC in liver has distinct clinicopathological characteristics.EBER positive staining is typicaly detected in LEL-CC,but not in LEL-HC.The 2 patients with LEL-CC are more likely to have a favorable prognosis than those 3 with LEL-HC.However,further studies with more cases are necessary to clarify the outcomes due to the limited cases currently available.