Clinical characteristics and prognosis of intraductal papillary mucinous neoplasm of the biliary tract

Objective: To study the clinical features, diagnosis, surgery treatment and prognosis of intraductal papillary mucinous neoplasm of the biliary tract (IPMN-B). Methods: The data of 16 patients with IPMN-B admitted to The First Hospital of Jilin University and Xi'an Third Hospital from January 2014 to January 2018 were retrospectively analyzed. Kaplan-Meier method was used to conduct the survival analysis. These patients included 10 males and 6 females, the median age was 57 years. Results: Clinical manifestations were mainly jaundice (11 cases), upper abdominal pain (12 cases) and hyperpyretic chills (4 cases), combined with bile duct stones (14 cases) and hepatic lobe atrophy (2 cases). The average size of the tumor was (2.6±0.7) cm. All of 16 cases were diagnosed as IPMN-B, including 5 cases of invasive carcinoma (4 cases were perineural invasion) without vascular invasion and 6 cases of non-invasion carcinoma. The pathological type included 8 cases of pancreaticobiliary duct type, 5 cases of gastric type, 2 cases of intestinal type and 1 case of eosinophils type. Laboratory tests showed abnormal liver function (12 cases), increased direct bilirubin (9 cases), increased carbohydrate antigen199 (CA199, 8 cases) and carcinoembryonic antigen (CEA, 4 cases). The enhanced CT detection showed 9 cases of intrabile duct mass and 14 cases of bile duct dilatation. Surgical resection is the main treatment method, including 2 cases of partial resection of liver, 12 cases of bile duct mass resection combined with choledochojejunostomy and 2 cases of pancreatoduodenectomy, all of whom achieved R0 resection. Fifteen patients were followed up and 4 died during the period. The median postoperative progression free survival was 31 months (95% CI: 33-47 months), and the recurrence rate at 1 year, 2-years and 3-years were 6.7%, 40.0% and 73.3%, respectively. The median overall survival was 35 months (95% CI: 23-47 months), and the 1 year, 2-years, and 3-years cumulative survival rates were 100%, 80.0%, and 53.3%, respectively. Conclusions: IPMN-B is a rare tumor of biliary tract system, which is difficult to be diagnosed early. The main treatment is surgical resection, which can achieve a good prognosis.