Khaderi Saira A, Sussman Norman L
Baylor College of Medicine, 6620 Main Street #1425, Houston, TX, 77030, USA.
Curr Gastroenterol Rep. 2015 Apr;17(4):17. doi: 10.1007/s11894-015-0438-0.
Primary sclerosing cholangitis (PSC) is a frequently progressive and fatal disease. Death from cancer occurs in a significant subset of patients with PSC. Patients with PSC have a 10 to 15 % lifetime risk of developing cholangiocarcinoma (CCA). About one third of CCAs are present in the first year after a diagnosis of PSC; the remainder are present with a frequency of about 1.5 % each year. Patients with concomitant PSC and inflammatory bowel disease (IBD) have a 4-fold higher risk of colorectal cancer (CRC) than patients with IBD alone and a 10-fold higher risk of CRC than the general population. The risk does not diminish with liver transplantation. This patient population also has a high frequency of carcinoma in gallbladder mass lesions. The risk for hepatocellular carcinoma (HCC) in the presence of cirrhosis is uncertain-two large cohort studies suggest that HCC is not as common as in other causes of cirrhosis. Although AASLD guidelines do not recommend routine screening for liver tumors in patients with PSC, we recommend MRI/MRCP and serum CA 19-9 levels in patients with PSC every 6 months to screen for CCA, HCC, pancreatic cancer, and gallbladder cancer. Screening colonoscopy at the diagnosis of PSC and surveillance colonoscopies every 1-2 years should be performed in those with PSC and IBD.
原发性硬化性胆管炎（PSC）是一种常呈进行性发展且可致命的疾病。相当一部分PSC患者会死于癌症。PSC患者一生中患胆管癌（CCA）的风险为10%至15%。约三分之一的CCA在PSC诊断后的第一年内出现；其余的每年出现频率约为1.5%。同时患有PSC和炎症性肠病（IBD）的患者患结直肠癌（CRC）的风险比仅患有IBD的患者高4倍，比普通人群高10倍。这种风险不会因肝移植而降低。该患者群体胆囊肿块病变中癌的发生率也很高。肝硬化患者发生肝细胞癌（HCC）的风险尚不确定——两项大型队列研究表明，HCC不像其他肝硬化病因那样常见。虽然美国肝病研究学会（AASLD）指南不建议对PSC患者进行肝脏肿瘤的常规筛查，但我们建议PSC患者每6个月进行一次MRI/MRCP检查和检测血清CA 19-9水平，以筛查CCA、HCC、胰腺癌和胆囊癌。对于患有PSC和IBD的患者，在诊断PSC时应进行结肠镜检查，并每1至2年进行一次结肠镜监测。
