Intrahepatic cholangiocarcinoma and hepatolithiasis: an unusual association in Western countries

Abstract

Hepatolithiasis is uncommon in Western countries and the relationship with cholangiocarcinoma is unusual. We report the association of hepatolithiasis and a cholangiocarcinoma in a Caucasian patient with a 17-year history of recurrent pancreatitis associated with hepatolithiasis. We discuss work-up and surgical treatment, and stress the need to keep in mind the possible association between hepatolithiasis and cholangiocarcinoma even in Western countries.

Indications and therapeutical options in hepatolithiasis

“…This disease is more common in Asia and is rare in Spain (2,3,8,12). Complications can occur if HL remains untreated (4,12), and their associated morbidity and mortality is high, oscillating between 1 and 10% (2,(13)(14)(15).…”

“…The co-existence of HL and cholangiocarcinoma varies between 0.36 and 13% of patients (1,5,17). It has been suggested that the mechanical irritation caused by hepatolithiasis, as well as chronic biliary infection and cholestasis, damages the biliary epithelium to the point of causing a tumor (5,8,10,18). Doubt always exists regarding the order in which these two diseases appear, whether the stenosis caused by the tumor induces HL or viceversa.…”

“…Patients with HL and cholangiocarcinoma are usually women with small tumors located in the left lobe and who present a higher frequency of episodes of cholangitis (17). Concomitant HL makes a correct preoperative diagnosis difficult (5,8,10,18). Postoperative morbidity in patients with cholangiocarcinoma is higher when a concomitant HL is present (8).…”

“…Concomitant HL makes a correct preoperative diagnosis difficult (5,8,10,18). Postoperative morbidity in patients with cholangiocarcinoma is higher when a concomitant HL is present (8). The 5-year survival of patients with HL and cholangiocarcinoma varies between 3 and 23%, and the presence of HL does not appear to alter survival (5,17).…”

“…It is a common disease in Southeast Asia and, in that region, is seen in 10-15% of patients with cholelithiasis (2-11); in contrast, it is very uncommon in Spain and other Western countries, where it is only observed in approximately 1% of these patients (8,12). Untreated HL can give rise to cholangitis, hepatic abscesses, biliary cirrhosis, portal hypertension, and cholangiocarcinoma (4,12).…”