Incidence of and risk factors for hepatocellular carcinoma in primary biliary cirrhosis: National data from Japan

Kenichi Harada,Toshiaki Nakano,Hirohito Tsubouchi,Yoshiyuki Ueno,Takafumi Ichida,Junko Hirohara,Yasuni Nakanuma,Yuko Kakuda

doi:10.1002/hep.26176

Abstract

Primary biliary cirrhosis (PBC) primarily affects females and is rarely complicated by hepatocellular carcinoma (HCC). Although the HCC incidence in PBC patients is low, several characteristics and risk factors associated with its development have been reported. In this study, national data concerning the current status of carcinogenesis in PBC patients in Japan are reviewed. Using data from two national questionnaire surveys, we investigated the clinicopathological findings associated with HCC in PBC patients. According to the data of all reviewed PBC patients, the HCC incidence was 2.4% (71/2946). The HCC incidence by gender was 5.1% (19/370) in males and 2.0% (52/2576) in females, and the proportion of males was 26.7%. Prognosis was significantly poorer in the PBC patients with HCC than in those without. Multivariate analysis of risk factors associated with HCC by gender revealed histological stage at the time of PBC diagnosis as an independent risk factor associated with the development of HCC in females, but not in males. Furthermore, data from another national survey of 178 PBC patients with HCC (male/female = 49/129; proportion of males 27.5%) revealed that the duration between the diagnosis of PBC and that of HCC was significantly shorter in males than in females. In addition, histological stage at the time of HCC diagnosis was an independent risk factor for HCC in females, whereas no risk factors were identified in males. these data indicate that males are at risk of developing HCC at any histological stage of PBC. Therefore, male PBC patients in particular should be carefully screened for HCC from the early stages of PBC.

Highlights

Primary biliary cirrhosis (PBC) primarily affects middle-aged females
We encountered PBC patients with hepatocellular carcinoma (HCC) during routine pathological assessments, and the number of these patients appears to have increased according to reports from other vi institutes.[11,17,18]
HCC is detected during follow-up for PBC, whereas some patients ew are simultaneously diagnosed with PBC and HCC or diagnosed with HCC prior to PBC

Summary

Introduction

Primary biliary cirrhosis (PBC) primarily affects middle-aged females. Histologically, the interlobular bile ducts are primarily damaged and show characteristic findings such as chronic nonsuppurative destructive cholangitis (CNSDC) followed by progressive bile duct loss.[1,2] A terminal feature of PBC is irreversible biliary cirrhosis, and liver transplantation is the sole treatment for hepatic failure.[3]. A comparative analysis of PBC patients with and without HCC revealed male gender, old age, low serum albumin levels, low serum total cholesterol levels, advanced histological stage, and symptomatic status at the time of PBC diagnosis as significant risk factors for HCC (Table 1).

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Conclusion