He Cai, Yin Hong-fang, Liu Ping, Zhang Ying, Zhang Jian-bo
Department of Pathology, Peking University First Hospital, Beijing 100034, China.
Department of Pathology, Peking University First Hospital, Beijing 100034, China. E-mail:
Zhonghua Bing Li Xue Za Zhi. 2013 Dec;42(12):824-8.
To investigate clinicopathological features of combined hepatocellular-cholangiocarcinoma (C-HCC-CC) with neuroendocrine carcinoma (NEC) differentiation and to review the literature.
The clinical data, histological manifestations and immunohistochemical staining results of two cases of C-HCC-CC were analyzed along with a review of the current literature.
Both patients were male with an average age of 57.5 years. Both patients were positive for hepatitis B virus antigen. The tumors of both cases demonstrated the following 3 unequivocal mixed elements: (1) polygonal epithelial tumor cells growing in nests or trabeculae with positive staining for Hepatocyte and AFP, diagnostic of hepatocellular carcinoma (HCC). Cytoplasmic bile production was present in the tumor cells in one case; (2) elliptic or short spindle-shape small blue tumor cells growing in nests or organoid pattern with Syn/CgA/CD56 positivity confirming the presence of neuroendocrine carcinoma (NEC) component; (3) oval tumor cells growing in nests or glandular forms with positivity of CK19 and CK7 confirming differentiation of cholangiocarcinoma (CC). In both cases, the tumors contained at least 20% of each of HCC, NEC and CC components.
C-HCC-CC with NEC is a rare form of primary malignancy of the liver with a poor prognosis.
探讨具有神经内分泌癌（NEC）分化的肝细胞-胆管细胞癌（C-HCC-CC）的临床病理特征并复习相关文献。
分析2例C-HCC-CC患者的临床资料、组织学表现及免疫组化染色结果，并复习当前文献。
2例患者均为男性，平均年龄57.5岁。2例患者乙肝病毒抗原均为阳性。2例肿瘤均呈现以下3种明确的混合成分：（1）多边形上皮肿瘤细胞呈巢状或小梁状生长，肝细胞和甲胎蛋白染色阳性，诊断为肝细胞癌（HCC）。1例肿瘤细胞胞质内有胆汁生成；（2）椭圆形或短梭形小蓝肿瘤细胞呈巢状或器官样生长，突触素/嗜铬粒蛋白A/CD56阳性，证实存在神经内分泌癌（NEC）成分；（3）椭圆形肿瘤细胞呈巢状或腺管状生长，细胞角蛋白19和细胞角蛋白7阳性，证实为胆管癌（CC）分化。2例肿瘤中，HCC、NEC和CC成分各至少占20%。
伴有NEC的C-HCC-CC是一种罕见的原发性肝脏恶性肿瘤，预后较差。
