目的

通过大样本手术切除病例分析，探讨胆管内乳头状肿瘤（IPNB）的临床特征和治疗策略。

方法

回顾性分析2006 年9 月至2022 年10 月，国内2 个大型医疗中心收治的94 例手术切除IPNB 患者资料。结果 （1）IPNB 术前仅21.3%（20/94）正确诊断，38.3%（36/94）术前诊断为胆管癌，MRI（MRCP）术前诊断正确性相对较高（24.1%，13/54）。典型影像学表现为胆管扩张合并胆管内肿物，新发现胆总管末端鸟嘴样狭窄也是一个重要特征。（2）38 例（40.4%）肿瘤位于左半肝，为最常见发病部位；IPNB 伴低级别、高级别上皮内瘤变各15 例（16.0%）、33 例（35.1%），为良性肿瘤；浸润性癌46 例（48.9%），为恶性肿瘤；肿瘤部位、多发肿瘤、分泌黏液与IPNB 良恶性无关。（3）患者均手术切除，其中肝切除72 例，肝外胆管切除合并胆肠吻合9 例，Whipple 13 例，R0 切除率为95.7%；61 例行淋巴结清扫，淋巴结阳性率3.3%（2/61）。（4）术后1、3、5、10 年生存率分别为91.1%、82.0%、73.5%、44.0%；单因素分析显示体质量是否下降、CA125 是否升高、肿瘤良恶性与患者远期预后有关（P＜0.05）；多因素分析显示恶性肿瘤是远期预后的独立危险因素（OR=2.479，95% CI：1.127～5.453，P=0.024）。与良性IPNB 相比，恶性IPNB 的中位生存时间更短（14.3 年vs 8.4 年，P＜0.05）。

结论

IPNB 术前难以正确诊断，近半数为浸润性癌，淋巴结阳性率低；术前需明确胆管内肿物位置及病变范围，必要时术中胆道镜检查；注意留取胆管切缘术中冰冻活检，以达到根治性切除；术后可选择性辅助治疗；IPNB 远期预后良好，恶性肿瘤是其独立危险因素。

Objective

To analyze the clinical characteristics and treatment strategies of intraductal papillary neoplasm of the bile duct （IPNB） through a large sample case analysis.

Methods

A retrospective analysis was conducted of the data of 94 patients with IPNB， admitted to two large medical centers in China from September 2006 to October 2022.

Results

（1） Only 21.3% （20/94） of IPNB patients were correctly diagnosed before surgery， and 38.3% （36/94） were misdiagnosed as cholangiocarcinoma.Compared to other preoperative examinations， MRI/MRCP had a higher diagnostic accuracy of 24.1% （13/54）.The typical imaging manifestation was bile duct dilation combined with intraductal tumor， and the newly recognized finding of specific characteristic as bird's beak like stenosis at the end of the common bile duct might be an important feature.（2） 38 cases of tumors were located in the left liver， accounting for 40.4%， which is the most common location.Pathologically， IPNB with low-grade intraepithelial neoplasia were 15 cases （16.0%），high-grade intraepithelial neoplasia were 33 cases （35.1%）， and invasive cancer were 46 cases （48.9%）.The first two were defined as benign tumors， while the latter was defined as malignant tumors.The location of the tumor and whether it secretes mucus were not related to the malignancy of IPNB.（3） All patients underwent surgical resection， including 72 cases of liver resection， 9 cases of extrahepatic bile duct resection combined with biliary intestinal anastomosis， and 13 cases of Whipple.The R0 resection rate was 95.7%， with 61 cases undergoing lymph node dissection and the lymph node positivity rate was 3.3% （2/61）.（4） The postoperative 1-， 3-， 5-， and 10-year survival rates were 91.1%， 82.0%， 73.5%， and 44.0%， respectively.Univariate analysis showed that weight loss， CA125 elevation， tumor malignancy were related to long-term prognosis of patients （P＜0.05）.Multivariate analysis showed that tumor malignancy alone was an independent risk factor for long-term prognosis （OR=2.479， 95% CI： 1.127-5.453， P=0.024）.Compared with benign IPNB，malignant IPNB had shorter survival time， with median survival time of 14.3 and 8.4 years， respectively（P＜0.05）.

Conclusions

IPNB is difficult to be diagnosed correctly before surgery， with nearly half being invasive cancer and a low lymph node positivity rate.Radical resection surgery should clarify the extent of the lesion before surgery， and if necessary， intraoperative cholangioscopy needs to be performed.In order to attain radical resection， the cutting edge of the bile duct should be provided for intraoperative frozen biopsy.Selective adjuvant treatment can be performed after surgery.The long-term prognosis of IPNB is good， and tumor malignancy is an independent risk factor.