胆管导管内乳头状黏液瘤的诊断和外科治疗

Diagnosis and surgical treatment of intraductal papillary mucinous neoplasm of the biliary tract

目的探讨胆管导管内乳头状黏液瘤（BT-IPMN）的临床特点、外科诊断和治疗方法。方法对2012年6月至2016年7月收住北京协和医院的19例BT-IPMN患者的临床资料进行回顾性分析。结果19例患者占同期胆管肿瘤患者（815例）的2．3％。其中男性9例，女性10例，男女比例1．0：1．1。年龄25-78岁，平均（60．6±12．9）岁。黄疸（10例）和腹痛腹胀（6例）是较常见的发病症状。术前较典型的影像学检查结果为胆管扩张和胆管肿物。所有患者均有病理诊断证实为胆管导管内乳头状黏液瘤。18例在我院接受手术治疗，手术方式包括左肝外侧叶或左半肝切除术8例、胰十二指肠切除术6例、高位胆管病变切除＋肝门胆管成型＋胆管空肠吻合术3例、中肝肿物切除术l例。平均手术时间280min，平均术中出血量515ml。14例患者行淋巴结清扫术，淋巴结阳性比例为3．2％（3／94）。获得随访16例，随访时间1-51个月，平均（25．7±19．5）个月，14例生存，2例死亡。死亡时间分别为术后3个月和17个月，死亡原因均为肿瘤复发转移。结论BT-IPMN临床少见，早期诊断较困难，淋巴结转移率低，手术是首选治疗方式并且具有较好的预后。

Objective To study the clinical features of intraductal papillary mucinous neoplasm of the biliary tract （BT-IPMN） and to analyse the diagnostic and surgical patterns. Method The data of 19 patients with BT-IPMN admitted from Jun. 2012 to Jul. 2016 were retrospectively analyzed. Results These 19 （2.3%） patients with BT-IPMN came from 815 patients with biliary tract tumors who were treated in our institution. There were 9 males and 10 females. The male to female ratio was 1.0： 1.1. The mean age was 60.6 ± 12.9 years with a range from 25 to 78 years. Jaundice （ in 10 patients） and abdominal discomfort （ in 6 patients） were the most common presenting symptoms. Bile duct dilatation and intraluminal mass were typical preoperative imaging findings. All these 19 patients were diagnosed to have BT-IPMN histopathologi- cally. 18 patients underwent surgery in our hospital. Left lateral hepatic sectionectomy or left hepatectomy was performed in 8 patients, pancreaticoduodenectomy in 6, local excision of bile duct and cholangiojejunos- tomy in 3 and central hepatectomy in 1. The average operating time was 280 minutes and the average amount of intra-operative bleeding was 515 ml. Fourteen patients underwent regional lymph node dissection and 3 positive lymph nodes were detected out of 94 resected lymph nodes. Sixteen patients were followed up from 1 to 51 months with a mean of （25.7 ± 19.5） months. Fourteen of these patients were still surviving. Two patients died 3 and 17 months after operation due to the tumor. Conclusions Intraductal papillary mucinous neoplasm of the biliary tract was very rare. Early diagnosis was not easy. There was a low percentage of lymphatic metastasis. Surgery was the first choice of treatment and complete resection of BT-IPMN was associated with good long-term survival.