探讨胆管内乳头状黏液瘤（IPMN-B）临床病理特征及诊治要点。

回顾性分析2017年12月至2020年1月湘雅常德医院收治的3例IPMN-B患者临床资料。患者均为女性，年龄分别为60、45、66岁。腹痛2例，黄疸1例，低热、纳差1例。TB升高2例，CA19-9升高1例。超声、CT、MRI检查示胆管扩张，胆管下端梗阻，胆管壁增厚。结合国内外文献总结IPMN-B的临床表现、病理特点、诊断、治疗及预后。

3例IPMN-B在胆道镜下均表现为胆管内壁上绒毛状或鱼卵状新生物，均行手术切除治疗。例1术后病理学检查为IPMN-B并中分化腺癌，出院后8个月出现胸背部及腰腿疼，复查CA19-9 1 614 kU/L；出院后10个月复查脊柱及骨盆MRI提示胸骨、锥体、骨盆、股骨多发异常信号，考虑转移；PET-CT示胆肠吻合口代谢活跃、全身多发骨质破坏，代谢活跃，考虑肿瘤复发、术区及全身骨多发转移；予以FOLFOX4方案化疗8次；术后18个月死亡。其余2例患者随访期间无复发，无消化道相关症状。

IPMN-B的临床表现与血生化检查指标不具有特异性，术前诊断主要以影像学为主，术中胆道镜准确判定病变范围，采取积极合理的手术治疗对IPMN-B的诊治十分重要。

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.