To explore clinical diagnosis, treatment and prognosis of patients with intrahepatic hepatolithiasis associated with cholangiocarcinoma (IHHCC).

Clinical data of 32 patients with IHHCC receiving operative treatment in the Affiliated Hospital of North Sichuan Medical College between December 2008 and December 2016 were retrospectively analyzed. The informed consents of all patients were obtained and the local ethical committee approval was received. There were 13 males and 19 femaleswith the age of 38-73 years and the median age of 61 years. The clinical pathological characteristics, diagnosis, treatment and prognosis of the patients were analyzed.

Significant weight loss was observed in 11 cases before being hospitalized. Eighteen cases had a history of illness for over 10 years. Twenty-four cases had a history of biliary operation, with the shortest operative history of 6 months and the longest 30 years. Moderate anemia was observed in 8 cases, severe anemia in 1 case, elevated CA19-9 in 20 cases before operation. Intrahepatic hepatolithiasis associated with space occupying focus was observed in13 cases by ultrasonography. Nineteen cases were diagnosed as IHHCC by abdominal CT, MRI and MRCP. Thepre-operative detection rate was 59%(19/32). Thirty cases underwent hepatectomy, 2 underwent liver tumor biopsy, and the post-operative pathological examinations indicated all were cholangiocarcinoma. Two patients undergoing liver tumor biopsy died of tumor metastasis and hepatic failure 2 months after surgery. Only 1 patient lived for more than 5 years after surgery, and the 1-, 3-, 5-year survival rate was respectively 38%, 16% and 3%.

For long-term intrahepatic hepatolithiasis patients, bile duct tumor should be alert when suffering from emaciation, anemia and elevated CA19-9. The pre-operative detection rate is low, and diagnosis mainly depends on imageological examination. Surgical resection is still the main treatment option while the prognosis is relatively poor.