Deutsch Melanie, Papatheodoridis George V, Tzakou Anastasia, Hadziyannis Stephanos J
Academic Department of Internal Medicine Hippocration General Hospital, Athens, Greece.
Eur J Gastroenterol Hepatol. 2008 Jan;20(1):5-9. doi: 10.1097/MEG.0b013e3282f163ed.
In primary biliary cirrhosis (PBC), the development of hepatocellular carcinoma (HCC) was thought to represent a rare complication. In contrast, extrahepatic malignancies have been reported to be significantly associated with PBC. The aim of this study was to determine the incidence of HCC and of extrahepatic malignancies in a large cohort of patients with PBC.
A total of 212 patients with documented PBC (19 men and 193 women) were followed up for a median of 6 (range, 1-23) years.
In total, 23 (10.8%) cases of malignancy were diagnosed; eight (3.8%) patients with HCC and 15 (7.0%) with extrahepatic malignancies. PBC patients were found to have a 10-year risk of 4% for developing HCC and of 13% for developing extrahepatic malignancies. The risk for HCC was significantly higher in the PBC patients with cirrhosis (15% at 10 years of follow-up). In contrast, the histologic stage of PBC does not influence the risk for extrahepatic malignancy.
Our study confirms that there is a risk of HCC in Greek patients with PBC, particularly in patients with stage IV PBC. The risk of extrahepatic malignancies is higher than that of HCC, but it is not influenced by the histologic stage of the liver disease.
在原发性胆汁性肝硬化（PBC）中，肝细胞癌（HCC）的发生被认为是一种罕见的并发症。相比之下，已有报道称肝外恶性肿瘤与PBC显著相关。本研究的目的是确定一大群PBC患者中HCC和肝外恶性肿瘤的发生率。
共有212例有记录的PBC患者（19例男性和193例女性）接受了中位时间为6年（范围1 - 23年）的随访。
总共诊断出23例（10.8%）恶性肿瘤；8例（3.8%）为HCC患者，15例（7.0%）为肝外恶性肿瘤患者。发现PBC患者发生HCC的10年风险为4%，发生肝外恶性肿瘤的风险为13%。肝硬化的PBC患者发生HCC的风险显著更高（随访10年时为15%）。相比之下，PBC的组织学分期不影响肝外恶性肿瘤的风险。
我们的研究证实，希腊PBC患者存在发生HCC的风险，尤其是IV期PBC患者。肝外恶性肿瘤的风险高于HCC，但不受肝病组织学分期的影响。
