Hall Catherine, McLaren Mairi, Mosse Charles
Division of Surgery, The Canberra Hospital, Garran, Australia.
J Surg Case Rep. 2022 Feb 16;2022(2):rjac018. doi: 10.1093/jscr/rjac018. eCollection 2022 Feb.
Carbohydrate antigen 19-9 (CA 19-9) is a specific tumour marker for pancreato-biliary malignancy. Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated condition in which IgG4 deposits infiltrate various organs, including the biliary tract manifesting IgG4 sclerosing cholangitis and pseudotumours. An 83-year-old woman presented with severe obstructive jaundice, weight loss and an extreme elevation of CA 19-9 level of 3295 kU/L. Magnetic resonance cholangiopancreatography (MRCP) revealed a pancreatic mass amputating the biliary tree. Liver function tests revealed a cholestatic pattern and severe hyperbilirubinaemia (289umol/L). IgG4 level was found to be high at 7.97 g/L. After treatment with high-dose corticosteroids, repeat MRCP 2 months later revealed disappearance of the pancreatic mass. There was also normalization of the bilirubin and a dramatic decrease of CA 19-9. This case reports the highest published benign elevation of Ca19.9 level in the setting of IgG4 disease. Differentiation between cholangiocarcinoma and IgG4-RD is important, as the treatment is vastly different.
糖类抗原19-9（CA 19-9）是胰胆管恶性肿瘤的一种特异性肿瘤标志物。免疫球蛋白G4相关疾病（IgG4-RD）是一种免疫介导的疾病，其中IgG4沉积物浸润包括胆道在内的各种器官，表现为IgG4硬化性胆管炎和假瘤。一名83岁女性出现严重阻塞性黄疸、体重减轻，CA 19-9水平极度升高至3295 kU/L。磁共振胰胆管造影（MRCP）显示胰腺肿块截断胆管树。肝功能检查显示胆汁淤积模式和严重高胆红素血症（289μmol/L）。发现IgG4水平较高，为7.97 g/L。经大剂量皮质类固醇治疗后，2个月后复查MRCP显示胰腺肿块消失。胆红素也恢复正常，CA 19-9显著下降。本病例报告了IgG4疾病背景下已发表的Ca19.9水平最高的良性升高情况。胆管癌与IgG4-RD的鉴别很重要，因为治疗方法差异很大。
