Imada J, Hoshino H, Nishimura D, Morita K, Yoshida N, Katada N, Sano H, Kato K, Mori N
Department of Internal Medicine, Koseiren Kamo Hospital, Toyota, Japan.
J Gastroenterol Hepatol. 1996 Jun;11(6):546-50. doi: 10.1111/j.1440-1746.1996.tb01700.x.
We present a case of primary biliary cirrhosis associated with simultaneous triple cancers: a hepatocellular carcinoma and adenocarcinomas of the common bile duct and gall-bladder. A 70 year old Japanese woman, who had been diagnosed with primary biliary cirrhosis (stage 2 by Scheuer) 15 years before, was admitted to Koseiren Kamo Hospital in a comatose state. Laboratory data were as follows: the ammonia level was high (164.0 micrograms/dL), the antimitochondrial antibody showed a 320-fold increase, a high level of alpha-fetoprotein was indicated (2677 ng/mL), hepatitis B surface antigen was negative and hepatitis C antibody by enzyme immunoassay was negative, although a test for the RNA of hepatitis C virus by polymerase chain reaction was positive (10(3.5) copies/50 microL). The patient's condition gradually worsened and the patient died of liver failure. Autopsy showed triple cancers in the liver (hepatocellular carcinoma; trabecular type, moderately differentiated), the common bile duct (well-differentiated papillary adenocarcinoma) and the gall-bladder (well-differentiated papillary adenocarcinoma) with primary biliary cirrhosis (stage 4). Primary biliary cirrhosis has been believed to be a low risk for the development of hepatocellular carcinoma, despite the high risk of extrahepatic malignancy. The simultaneous occurrence of triple cancers with primary biliary cirrhosis, to the best of our knowledge, has never been reported. The present case may provide additional evidence for a predisposition to malignancy in primary biliary cirrhosis.
肝细胞癌以及胆总管和胆囊腺癌。一名70岁的日本女性,15年前被诊断为原发性胆汁性肝硬化(Scheuer分期2期),以昏迷状态入住小泉礼仁加茂医院。实验室检查数据如下:氨水平升高(164.0微克/分升),抗线粒体抗体升高320倍,甲胎蛋白水平较高(2677纳克/毫升),乙肝表面抗原阴性,酶免疫法检测丙肝抗体阴性,尽管聚合酶链反应检测丙肝病毒RNA呈阳性(10(3.5)拷贝/50微升)。患者病情逐渐恶化,最终死于肝功能衰竭。尸检显示肝脏(肝细胞癌;小梁型,中度分化)、胆总管(高分化乳头状腺癌)和胆囊(高分化乳头状腺癌)存在三种癌症,同时伴有原发性胆汁性肝硬化(4期)。尽管原发性胆汁性肝硬化发生肝外恶性肿瘤的风险较高,但一直被认为发生肝细胞癌的风险较低。据我们所知,原发性胆汁性肝硬化同时发生三种癌症的情况从未有过报道。本病例可能为原发性胆汁性肝硬化易于发生恶性肿瘤提供更多证据。