肝内胆管囊腺瘤和囊腺癌的临床分析

Clinical analysis of intrahepatic biliary cystadenoma and cystadenocarcinoma

  • 摘要: 目的:总结肝内胆管囊腺瘤和囊腺癌的诊断与治疗经验及影响预后的因素。
    方法:回顾性分析2001年1月至2012年12月第二军医大学附属东方肝胆外科医院经病理学检查证实的46例肝内胆管囊腺瘤和19例肝内胆管囊腺癌患者的临床资料。患者入院行B超、CT与MRI检查。采用Pringle法或不阻断肝门的方法施行肝切除术,对预后因素进行分析。采用电话或信函随访,随访时间截至2013年4月。正态分布的计量资料用〖AKx-D〗±s表示;采用KaplanMeier法计算生存率,生存率的比较采用Logrank法检验。单因素分析采用χ2检验。多因素分析采用COX回归模型。结果:(1)影像学表现: B超、CT或MRI检查提示肝内胆管囊腺瘤和囊腺癌影像学表现类似,囊壁有乳头状突起,部分囊壁明显增厚,囊腔可呈多房性。术前影像学检查确诊肝内胆管囊腺瘤20例,肝内胆管囊腺癌7例。(2)手术情况:46例肝内胆管囊腺瘤患者中,行肿瘤局部剜除术22例,肝左叶切除术15例,肝左外叶切除术3例,右半肝切除术4例,肝右后叶切除术1例,肝右前叶切除术1例。38例采用Pringle法阻断肝门,8例未行肝门阻断。手术时间为(175±70)min,术中出血量平均为300 mL(200~400 mL),术后发生胆汁漏4例,再出血3例,肝功能不全2例,均经保守治疗后好转。19例肝内胆管囊腺癌患者中,行肿瘤局部剜除术9例,肝左叶切除术8例,肝左外叶切除术1例,肝右后叶切除术1例。13例采用Pringle法阻断肝门,6例未行肝门阻断。手术时间为(210±68)min,术中平均出血量为500 mL(200~800 mL),术后发生胆汁漏3例, 再出血2例,肝功能不全1例,经保守治疗后好转。术后病理学检查:肝内胆管囊腺瘤囊腔内表层多为单层柱状上皮,排列整齐,无异型;肝内胆管囊腺癌囊腔内多有乳头状突起,细胞异型明显,并向基底膜浸润。(3)随访情况:57例患者获得随访,8例失访(囊腺瘤5例、囊腺癌3例)。随访时间为14~139个月,其中肝内胆管囊腺瘤2例复发(1例因术后肝衰竭死亡);肝内胆管囊腺癌13例复发,10例死亡,其中1例为肝内胆管囊腺瘤恶变。(4)预后因素分析:肝内胆管囊腺瘤患者1、3、5年生存率均为98%。肝内胆管囊腺癌患者的1、3、5年生存率分别为88%、31%、13%。 单因素分析结果显示:CA19-9是影响肝内胆管囊腺癌患者的无瘤生存率和总体生存率的危险因素(χ2=8.540,4.946,P<0.05)。多因素分析结果显示:CA19-9升高是影响肝内胆管囊腺癌患者的无瘤生存率和总体生存率的独立危险因素(OR=8.239,5.365,95%可信区间:1.664~40.800,1.022~28.172,P<0.05)。
    结论:肝内胆管囊腺瘤与囊腺癌临床症状不典型,影像学检查有助于术前评估,但符合率低。治疗以手术切除为主。CA19-9是评估肝内胆管囊腺癌预后的重要指标。

     

    Abstract: Objective:To summarize the diagnosis and treatment experiences of intrahepatic biliary cystadenoma and cystadenocarcinoma and the factors affecting prognosis of patients.
    Methods:The clinical data of 46 patients with cystadenoma and 19 patients with cystadenocarcinoma who were admitted to the Eastern Hepatobiliary Surgery Hospital from January 2001 to December 2012 were retrospectively analyzed. All the patients underwent Bultrasound, computed tomography (CT) and magnetic resonance imaging (MRI) examinations, and received the resection of liver by the Pringle maneuver or without hepatic inflow occlusion, and then the prognoses of patients was analyzed. All the patients were followed up via telephone interview and correspondence up to April 2013. The measurement data with normal distribution were presented as 〖AKx-D〗±s. The survival curve was drawn by KaplanMeier method and survival analysis was done using the Logrank test. The univariate analysis and multivariate analysis were done using the chisquare test and COX regression model.
    Results:(1)The results of B ultrasound, CT and MRI showed that the imaging features of cystadenoma and cystadenocarcinoma were very similar, which included  papilla on the cystic wall, obvious thickening of cystic wall and multilocular cystic 〖HJ*4〗spaces. Before operation, cystadenoma was confirmed in 20 patients and cystadenocarcinoma in 7 patients by imaging examinations. (2)Fortysix patients with intrahepatic biliary cystadenoma underwent surgical treatment, including 22 with local tumor enucleation, 15 with left lobectomy, 3 with external lobectomy, 4 with right hemihepatectomy, 1 with right posterior lobectomy and 1 with right anterior lobectomy. There were 38 patients with the vascular inflow occlusion by the Pringle maneuver and 8 without vascular inflow occlusion. The operation time and mean volume of blood loss were (175±70)minutes and 300 mL (range, 200-400 mL). There were 4 patients with bile leakage, 3 with rebleeding and 2 with liver dysfunction, and they got full recovery by conservative treatment. Among 19 patients with intrahepatic biliary cystadenocarcinoma, 9 received local tumor enucleation, 8 received left lobectomy, 1 received left external lobectomy and 1 received right posterior lobectomy. There were 13 patients with vascular inflow occlusion by the Pringle maneuver and 6 without vascular inflow occlusion. The operation time and volume of blood loss were (210±68)minutes and 500 mL (range, 200-800 mL). After operation, the bile leakage was detected in 3 patients, rebleeding in 2 patients and liver dysfunction in 1 patient, and the conditions of patients were improved by conservative treatment. The results of postoperative pathological examinations showed that intrahepatic biliary cystadenoma was arrangedirreguarly simple columnar epithelium without cytologic atypia in the cystic spaces and intrahepatic biliary cystadenocarcinoma was papilla with cytologic atypia and basement membrane infiltration in the cystic spaces. (3)Fiftyseven patients were followed up for 14-139 months and 8 were lost to followup (5 with cystadenoma and 3 with cystadenocarcinoma). During the followup, 2 patients had recurrence of intrahepatic biliary cystadenoma, and 1 of whom died of postoperative liver dysfunction. Among 13 patients with recurrence of intrahepatic biliary cystadenocarcinoma, 10 died and including 1 with canceration of cystadenoma. (4)The 1, 3, 5year survival rates of the patients with intrahepatic biliary cystadenoma and cystadenocarcinoma were 98%, 98%, 98% and 88%, 31%, 13%, respectively. CA19-9 was a risk factor affecting the tumorfree survival rate and overall survival rate in patients with intrahepatic biliary cystadenocarcinoma by the univariate analysis (χ2=8.540, 4.946, P<0.05). The high level of CA19-9 was an independent risk factor affecting the tumor free survival rate and overall survival rate in patients with intrahepatic biliary cystadenocarcinoma by the multivariate analysis(OR=8.239, 5.365, 95% confidence interval: 1.664-40.800, 1.022-28.172, P<0.05).
    Conclusions:The clinical symptoms of intrahepatic biliary cystadenoma and cystadenocarcinoma are atypical. Imaging examination can help improve to preoperative evaluation with the low coincidence rate. The surgical resection is a main treatment method. CA19-9 is an important index for evaluating the prognosis of patients with intrahepatic biliary cystadenocarcinoma.

     

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