胆管黏液腺癌13例临床特征和预后分析

Clinicopathological features and postoperative prognosis of mucinous cholangiocarcinoma in 13 cases

目的分析胆管黏液腺癌的临床病理学特征和预后特点。方法回顾性分析东方肝胆外科医院2002年1月—2014年12月间收治的13例胆管黏液腺癌患者的临床资料,总结其临床病理学特征、诊断和治疗策略,根据随访结果进行预后分析。根据有无淋巴结转移将患者分为两组并比较其预后的差异。结果患者自症状出现至手术的时间为7d^13年,中位时间为4个月。11例患者有临床症状,其中右上腹胀痛不适7例,发热3例,黄疸7例,乏力4例;无临床症状2例。实验室检查各项指标水平均升高。13例患者中,6例术前影像学检查见局部淋巴结转移。胆管黏液腺癌的大体病理学特征与其MRI检查表现相符。13例患者中,11例行根治性手术,其中5例肝内胆管黏液腺癌患者行肝切除术(1例伴肝门部淋巴结转移的患者行肝切除术+肝门部淋巴结清扫术);4例肝门部胆管黏液腺癌患者行肝门部胆管癌根治术(均行局部淋巴结清扫术);2例远端胆管黏液腺癌患者行胰十二指肠切除术;1例肝内胆管黏液腺癌患者因术中发现腹腔内肿瘤广泛转移而行右肝胆管癌栓取出、T管引流术;1例肝门部胆管黏液腺癌患者行胆囊和肝外胆管切除、肝门部淋巴结清扫、胆肠吻合术,术后病理学检查提示切缘阳性,术后辅以放射治疗。所有患者术后病理学检查均证实为胆管黏液腺癌,其中7例阻塞性黄疸患者术后病理学检查证实为胆管黏液性梗阻伴远端胆管扩张。13例患者均顺利出院,无围术期死亡。随访时间7~80个月,中位随访时间为33个月。截至2015年12月31日,有7例患者存活,生存时间7~80个月,中位生存时间为54个月,95%CI为27.02~80.98个月。采用Kaplan-Meier估计法得出术后1、3、5年患者的生存率分别为(92.3±7.4)%、(53.8±17.7)%、(35.9±18.8)%。分别对淋巴结转移组(6例)和无淋巴结转移组(7例)进行生存分析。因无淋巴结转移组的病例数少且数据中事件或死亡病例太少,大部分生存时间对应的生存率均>50%,中位生存时间不存在,同时也无法计算95%CI。淋巴结转移组生存时间7~31个月,中位生存时间为23个月,95%CI为0.1~45.9个月。采用Log-rank法比较两组生存率的差异有统计学意义(P=0.005),提示无淋巴结转移组的预后显著优于淋巴结转移组。结论扩张的胆管旁低密度占位和与胆管相通的多发囊性占位是胆管黏液腺癌显著的影像学特点,淋巴结转移是影响手术预后的危险因素。

Objective To analyze clinical characteristics and prognosis of mucinous cholangiocarcinoma （MCC）. Methods A retrospective study was made on clinical manifestations, operative procedures and postoperative prognosis in 13 cases oi MCC. The 13 patients were divided into two groups according to lymph node metastasis and the prognosis was compared between two groups. Results The median duration from the onset of symptoms to surgery was 4 months （range, 7 d-- 13 years）. Laboratory indicators were elevated in all patients. Clinical symptoms; right upper quadrant pain and discomfort in 7 cases, fever in 3 cases, jaundice in 7 cases, and fatigue in 4 cases. There were 2 cases without clinical symptoms. Preoperative imaging examination showed lymph node metastasis in 6 cases. Gross pathological features of mucinous cholangiocarcinoma were consistent with its MRI features. Eleven patients underwent radical surgery. Of them, 5 cases of intrahepatic mucinous cholangiocarcinoma underwent liver resection （1 case with hilar lymph node metastasis underwent hepatic resection＋ hilar lymph node dissection）, 4 cases of hilar mucinous cholangiocarcinoma underwent radical resection of hilar cholangiocarcinoma （all underwent lymph node dissection）, 2 cases of mucinous cholangiocarcinoma in the distal bile duct underwent pancreaticoduodenectomy. Extensive tumor metastasis was found during surgery in one case of intrahepatic mucinous cholangiocarcinoma, and tumor thrombus removal in right hepatic bile duct ＋ T tube drainage were performed. One case of hilar mucinous cholangiocarcinoma underwent gallbladder and extrahepatic bile duct resection ＋ hilar lymph node dissection ＋ cholangioenterostomy and continued with postoperative radiotherapy since the pathological examinations showed positive margins. All pathology specimens were confirmed as mucinous cholangiocarcinoma. Mucinous biliary obstruction with distal bile duct dilatation were pathologically proved in 7 patients with obstructive jaundice. All the 13 patients were discharged properly. The median follow-up time was 33 months （range, 7 to 80 months）. Till December 31, 2015, seven patients survived. The median survival time was 54 months （range, 7 to 80 months）, and 95% CI was 27.02 - 80.98 months. Survival rates of 1, 3, and 5 yeai＇s were （92.3 ± 7.4） %, （53.8 ± 17.7） %, and （35.9 ± 18.8） % calculated by Kaplan-Meier estimation method, respectively. Because of inadequate cases or death events in the non-lymph node metastasis group （n = 7）, the survival probability corresponding to most of the survival time were〉50%, the median survival time did not exist, and it was unable to calculate the 95% Cl. The median survival time was 23 months （range, 7 to 31 months）, and 95% CI was 0.1 to 45.9 months in lymph node metastasis group （n= 6）. Log-rank method found that there was significant difference in the survival rate between the two groups （ P = 0.005）, indicating prognosis in non-lymph node metastasis group was significantly better than lymph node metastasis group. Conclusion The masses of low density around outstretched bile duct and multiple mucinous cystic with bile duct communication are remarkable imaging features of mucinous cholangiocarcinoma. Lymph node metastasis is a risk factor for prognosis. （Shanghai Med J, 2016, 39= 675-679）