To investigate clinicopathological features, diagnosis and treatment of intraductal papillary mucinous neoplasm of the bile tract (IPMN-B).

Clinical data of 3 patients with IPMN-B admitted to Xiangya Changde Hospital from December 2017 to January 2020 were retrospectively analyzed. All patients were female, aged 60, 45 and 66 years. 2 patients experienced abdominal pain, 1 case of jaundice, 1 case of low fever and anorexia. TB levels were increased in 2 cases and CA19-9 level was increased in 1 case. Ultrasound, CT scan and MRI showed bile duct dilatation, obstruction at the lower end of bile duct and thickening of bile duct wall. Clinical manifestations, pathological features, diagnosis, treatment and prognosis of IPMN-B were summarized according to literature review at home and abroad.

All3 IPMN-B patients showed villous or fish-egg-like lesions on the inner wall of bile duct under choledochoscopy, and all of them were surgically removed. Case 1 was diagnosed with IPMN-B complicated with moderately-differentiated adenocarcinoma by postoperative pathological examination, and experienced chest, back, waist and leg pain at 8 months after discharge. CA19-9 level was detected as 1 614 kU/L. At 10 months after discharge, MRI of the spine and pelvis showed multiple abnormal signals in the sternum, vertebra, pelvis and femur. The possibility of metastasis was considered. PET-CT revealed active metabolism at biliary-intestinal anastomosis site, systemic bone destruction and active metabolism. The possibility of tumor recurrence and of surgical metastase site, systemic bone metastases was suspected. FOLFOX4 chemotherapy was given for 8 times. The patient died at 18 months after operation. No recurrence and digestive tract-related symptoms were reported in the other 2 cases during follow-up.

Clinical manifestations and biochemical detection indexes of IPMN-B are not specific. Preoperative diagnosis is mainly made based on imaging examination. Accurate delineation of lesion range by intraoperative choledochoscopy, and active and rational surgical interventions play a critical role in the diagnosis and treatment of IPMN-B.