布加综合征与肝内胆管细胞癌的不常见组合：病例报告及文献复习。
Budd Chiari Syndrome and Intrahepatic Cholangiocarcinoma, An Unusual Combination: Case Report and Review of the Literature.
机构信息
Department of Gastroenterology, All India Institute of Medical Sciences, New Delhi, India.
出版信息
Perm J. 2020 Nov;24:1-3. doi: 10.7812/TPP/19.204.
INTRODUCTION
Intrahepatic cholangiocarcinoma arising in the setting of Budd Chiari syndrome is uncommon and its prognostic and management implications differ from hepatocellular carcinoma.
CASE PRESENTATION
We report a case of intrahepatic cholangiocarcinoma in a patient with primary Budd Chiari syndrome. Hepatocellular carcinoma is known to occur with Budd Chiari syndrome. It was difficult to differentiate from hepatocellular carcinoma in the presence of increased alfa-fetoprotein levels. The contrast imaging showed features of progressive enhancement in the arterial, portal, and venous phases. A targeted liver biopsy showed histological features typical for cholangiocarcinoma. Immunostaining for cytokeratin 7 and cytokeratin 20 were positive, whereas that for arginase was negative, suggesting an intrahepatic cholangiocarcinoma. The patient was planned for inferior vena cava angioplasty followed by resection for intrahepatic cholangiocarcinoma.
CONCLUSION
Previously, only secondary Budd Chiari syndrome developing in the background of primary liver tumor has been described; no report exists of intrahepatic cholangiocarcinoma arising in background of primary Budd Chiari syndrome.
简介
在布加综合征背景下发生的肝内胆管细胞癌并不常见，其预后和治疗意义与肝细胞癌不同。
病例介绍
我们报告了一例原发性布加综合征患者的肝内胆管细胞癌。众所周知，乙型肝炎后会发生肝细胞癌。在甲胎蛋白水平升高的情况下，很难与肝细胞癌区分。对比成像显示动脉期、门脉期和静脉期进行性增强的特征。靶向肝活检显示出典型的胆管癌组织学特征。细胞角蛋白 7 和细胞角蛋白 20 的免疫染色阳性，而精氨酸酶染色阴性，提示为肝内胆管细胞癌。计划对下腔静脉进行血管成形术，然后对肝内胆管细胞癌进行切除术。
结论
以前，仅描述了原发性肝脏肿瘤背景下发生的继发性布加综合征；尚无原发性布加综合征背景下发生肝内胆管细胞癌的报道。
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