Abstract：
A 67-year-old man with a low-grade fever was found to have a 25-mm diameter tumor of the left hepatic umbilical portion. The tumor was accompanied by occlusion of the left portal vein. Positron emission tomography using fluorodeoxyglucose showed that the tumor had abnormally high metabolic activity. Magnetic resonance imaging revealed the left hepatic duct segmental narrowing. There was a mild elevation in serum IgG4 (206 mg/dL). Intrahepatic cholangiocarcinoma was suspected. Instead of planned hepatectomy, the patient was forced to undergo emergency surgery for biliary panperitonitis caused by intrahepatic bile duct rupture. Intraoperative ultrasonography revealed a hypoechoic tumor-like thickened Glissonean sheath and needle biopsy was performed. Histologic examination confirmed fibrous tissue with IgG4-positive plasma cell infiltration without neoplastic proliferation. He was diagnosed with IgG4-related sclerosing cholangitis (IgG4-SC) presenting hepatic inflammatory pseudotumor. After his general condition improved, he underwent left hepatectomy. Macroscopic findings showed extreme fibrosis of the Glissonean sheath of the umbilical portion, and diffuse granular lesion aggregated in the left lateral segment. Microscopic examination confirmed chronic cholangitis and dense portal fibrosis in the umbilical portion and diffuse xanthogranulomatous inflammation. This is the first case report of spontaneous rupture of the intrahepatic bile duct in patient with IgG4-SC.
摘要：
一名67岁的低烧男子被发现左肝脐部分有25毫米直径的肿瘤。肿瘤伴有左门静脉闭塞。使用氟脱氧葡萄糖的正电子发射断层扫描显示肿瘤具有异常高的代谢活性。磁共振成像显示左肝导管节段狭窄。血清IgG4轻度升高(206mg/dL)。怀疑肝内胆管癌。而不是计划的肝切除术，由于肝内胆管破裂引起的胆汁性腹膜炎，患者被迫接受急诊手术。术中超声检查显示低回声肿瘤样增厚的Glissonean鞘，并进行了穿刺活检。组织学检查证实纤维组织有IgG4阳性浆细胞浸润，无肿瘤增生。他被诊断为IgG4相关硬化性胆管炎（IgG4-SC），表现为肝脏炎性假瘤。一般情况好转后,他做了左肝切除术.宏观发现显示脐带部分的Glissonean鞘极度纤维化，和聚集在左外侧段的弥漫性颗粒性病变。显微镜检查证实慢性胆管炎和脐部致密门静脉纤维化和弥漫性黄色肉芽肿性炎症。这是IgG4-SC患者肝内胆管自发性破裂的首例报告。
