探讨肝内胆管导管内乳头状瘤（IPNB）恶变伴门静脉及胆道变异的诊治。

回顾性分析2020年12月南京医科大学附属苏州医院收治的1例肝内IPNB恶变伴门静脉及胆道变异患者临床资料。患者女，65岁，因"反复上腹部不适4年余"入院。体检无明显阳性体征，血常规、肝功能及肿瘤标志物均无明显异常。上腹部CT示肝左叶胆管明显扩张，左内叶胆管内可见高密度影，增强后明显强化；右后叶支胆管稍扩张。上腹部MRI见肝左内叶胆管内占位，增强后呈不均匀强化。入院诊断考虑肝内IPNB。三维重建示门静脉右前支汇入门静脉左支；右后叶支胆管汇入左肝管后，与右前叶支胆管组成肝总管。患者签署知情同意书，符合医学伦理学规定。于2020年12月11日在全身麻醉下行左半肝及Spiegel叶切除术。观察患者手术情况及术后疗效。

术中发现肿瘤主要位于左肝管内，并侵及右后叶支胆管开口。在门静脉右前支与左支汇合处远端离断门静脉左支，并于距离肿瘤1 cm处离断右后支胆管，左肝管则于汇入右前胆管处切断。两处胆管切缘分别送检，镜下未见异型细胞。行肝十二指肠韧带淋巴结清扫后缝合左肝管断端，右后叶胆管与空肠行Roux-en-Y吻合术。术后病理学检查示肝左叶IPNB，近肝门处肿瘤癌变；肝十二指肠韧带淋巴结未见癌转移，胆管切缘未见累及。患者恢复良好，术后8 d出院，随访至投稿日期，未见肿瘤复发及转移。

肝内IPNB是一种罕见的胆管肿瘤，临床表现及实验室检查均缺乏特异性，影像学和病理学检查有助于明确诊断。术前三维重建可发现血管和胆管变异，其对手术方案选择至关重要。早期施行根治性手术能显著改善患者预后。

To investigate the diagnosis and treatments of intrahepatic intraductal papillary neoplasm of bile duct (IPNB) complicated with portal vein and biliary tract variations.

Clinical data of 1 patient with malignant transformation of intrahepatic IPNB complicated with portal vein and biliary tract variations admitted to Suzhou Hospital Affiliated to Nanjing Medical University in December 2020 were retrospectively analyzed. A 65-year-old female patient was admitted to hospital due to "recurrent upper abdominal discomfort for more than 4 years". No obvious positive sign was found in physical examination. No evident abnormality was found in routine blood test, liver function and tumor markers. Significant dilated bile duct in the left lobe was observed by upper abdomen CT scan, and high-density shadow was observed in the bile duct of left inferior lobe, which was significantly intensified after enhancement. The bile duct of right posterior lobe was slightly dilated. A space-occupying lesion in the bile duct of left inferior lobe was detected by upper abdomen MRI, which was unevenly intensified after enhancement. The diagnosis of intrahepatic IPNB was considered upon admission. Three-dimensional reconstruction revealed that the right anterior branch of portal vein converged into the left branch of portal vein. The bile duct of right posterior lobe converged into the left hepatic duct, composing common hepatic duct with the bile duct of right anterior lobe. The informed consent of this patient was obtained and the local ethical committee approval was received. On December 11, 2020, the patient underwent left hemihepatectomy and Spiegel lobectomy under general anesthesia. The intraoperative conditions and postoperative efficacy were observed.

The tumor was found mainly located in the left hepatic duct and invaded into the opening of bile duct of right posterior lobe during the operation. The left branch of portal vein was ligated at the distal end of the junction of right anterior branch and left branch of portal vein, and the right posterior bile duct was resected at 1 cm from the tumor, and the left hepatic duct was resected at the junction of the right anterior bile duct. The margins of 2 bile duct samples were obtained for pathological examination, and no abnormal cells were detected under microscope. After lymph node dissection of hepatoduodenal ligament, the left hepatic duct was sutured. Roux-en-Y anastomosis was performed between the bile duct of right posterior lobe and jejunum. Postoperative pathological examination indicated IPNB in the left lobe, and malignant transformation was reported adjacent to the hilum. No cancer metastasis was found in the lymph nodes of hepatoduodenal ligament, and the incisional margins of bile duct were not involved. The patient recovered well, discharged at postoperative 8 d after operation. She was followed up postoperatively till the date of submission. No tumor recurrence or metastasis was found.

Intrahepatic IPNB is a rare bile duct tumor without specific clinical manifestation and laboratory index. Imaging and pathological examination contributes to confirming the diagnosis. Preoperative three-dimensional reconstruction can identify vascular and bile duct variations, which plays a significant role in the selection of surgical regimen. Early radical resection may significantly improve the clinical prognosis.