National guidelines for the diagnosis and treatment of hilar cholangiocarcinoma

A consensus meeting of national experts from all major national hepatobiliary centres in the country was held on May 26,2023,at the Pakistan Kidney and Liver Institute&Research Centre(PKLI&RC)after initial consultations with the experts.The Pakistan Society for the Study of Liver Diseases(PSSLD)and PKLI&RC jointly organised this meeting.This effort was based on a comprehensive literature review to establish national practice guidelines for hilar cholangiocarcinoma(hCCA).The consensus was that hCCA is a complex disease and requires a multidisciplinary team approach to best manage these patients.This coordinated effort can minimise delays and give patients a chance for curative treatment and effective palliation.The diagnostic and staging workup includes high-quality computed tomography,magnetic resonance imaging,and magnetic resonance cholangiopancreato-graphy.Brush cytology or biopsy utilizing endoscopic retrograde cholangiopancreatography is a mainstay for diagnosis.However,histopathologic confirmation is not always required before resection.Endoscopic ultrasound with fine needle aspiration of regional lymph nodes and positron emission tomography scan are valuable adjuncts for staging.The only curative treatment is the surgical resection of the biliary tree based on the Bismuth-Corlette classification.Selected patients with unresectable hCCA can be considered for liver transplantation.Adjuvant chemotherapy should be offered to patients with a high risk of recurrence.The use of preoperative biliary drainage and the need for portal vein embolisation should be based on local multidisciplinary discussions.Patients with acute cholangitis can be drained with endoscopic or percutaneous biliary drainage.Palliative chemotherapy with cisplatin and gemcitabine has shown improved survival in patients with irresectable and recurrent hCCA.

Diagnosis and surgical treatment of hepatic hilar cholangiocarcinoma

BACKGROUND: Hepatic hilar cholangiocarcinoma can be diagnosed early with the progress in diagnostic imaging, and thus the rate of resection of the tumor has increased markedly. To assess the effectiveness of resection, we reviewed 185 cases of hepatic hilar cholangiocarcinoma diagnosed and treated at our hospital. METHODS: The clinical data of 185 patients with hepatic hilar cholangiocarcinoma who had been treated surgically from 1972 to 2006 were retrospectively analyzed. RESULTS: The records of the 185 patients were divided into first stage (1972-1986) or second stage (1987-2006) according to the incidence of the tumor and its resection rate. Primary symptoms included upper abdominal discomfort or pain, anorexia, tiredness, weight loss and progressive jaundice. Ultrasonography, computed tomography (CT), magnetic resonance imaging (MRI), and magnetic resonance cholangiopancreatography (MRCP) were first line methods for atraumatic diagnosis. If the patients displayed intrahepatic bile duct dilatation or were diagnosed as suffering from extrahepatic obstructive jaundice, percutaneous transhepatic cholangiography (PTC), MRCP or endoscopic retrograde cholangiopancreatography (ERCP) should be used. In this series, 87 patients underwent resection of the tumor (47.0%). Of the 87 patients, 43 received radical resection and 44 palliative resection. Fifteen patients underwent resection in the first stage and 72 in the second stage. A total of 74 patients were followed up after the resection. The median survival time of the radical resection group was 37 months and that of the palliative resection group was 17 months (P<0.001). The other 62 patients receiving no resection died within 1.5 years. CONCLUSIONS: Once patients are diagnosed with hepatic hilar cholangiocarcinoma, they should undergo exploratory laparotomy. Resection is the most effective method for the treatment of hepatic hilar cholangiocarcinoma.

Preoperative biliary drainage in patients with hilar cholangiocarcinoma undergoing major hepatectomy

AIM:To investigate the effect of preoperative biliary drainage(PBD)in jaundiced patients with hilar cholangiocarcinoma(HCCA)undergoing major liver resections.METHODS:An observational study was carried out by reviewing a prospectively maintained database of HCCA patients who underwent major liver resection for curative therapy from January 2002 to December 2012.Patients were divided into two groups based on whether PBD was performed:a drained group and an undrained group.Patient baseline characteristics,preoperative factors,perioperative and short-term postoperative outcomes were compared between the two groups.Risk factors for postoperative complications were also analyzed by logistic regression test with calculating OR and 95%CI.RESULTS:In total,78 jaundiced patients with HCCA underwent major liver resection:32 had PBD prior to operation while 46 did not have PBD.The two groups were comparable with respect to age,sex,body mass index and co-morbidities.Furthermore,there was no significant difference in the total bilirubin(TBIL)levels between the drained group and the undrained group at admission(294.2±135.7 vs 254.0±63.5,P=0.126).PBD significantly improved liver function,reducing not only the bilirubin levels but also other liver enzymes.The preoperative TBIL level was significantly lower in the drained group as compared to the undrained group(108.1±60.6 vs 265.7±69.1,P=0.000).The rate of overall postoperative complications(53.1%vs 58.7%,P=0.626),reoperation rate(6.3%vs 6.5%,P=1.000),postoperative hospital stay(16.5 vs 15.0,P=0.221)and mortality(9.4%vs 4.3%,P=0.673)were similar between the two groups.In addition,there was no significant difference in infectious complications(40.6%vs 23.9%,P=0.116)and noninfectious complications(31.3%vs 47.8%,P=0.143)between the two groups.Univariate and multivariate analyses revealed that preoperative TBIL>170μmol/L(OR=13.690,95%CI:1.275-147.028,P=0.031),Bismuth-Corlette classification(OR=0.013,95%CI:0.001-0.166,P=0.001)and extended liver resection(OR=14.010,95%CI:1.130-173.646,P=0.040)were independent risk factors for postoperative complications.CONCLUSION:Overall postoperative morbidity and mortality rates after major liver resection are not improved by PBD in HCCA patients with jaundice.Preoperative TBIL>170μmol/L,Bismuth-Corlette classification and extended liver resection are independent risk factors linked to postoperative complications.

Endoscopic or percutaneous biliary drainage in hilar cholangiocarcinoma:When and how?

Hilar cholangiocarcinoma (hCCA) is a primary liver tumor associated with a dimprognosis. The role of preoperative and palliative biliary drainage has long beendebated. The most common techniques are endoscopic retrograde cholangiopancreatography(ERCP) and percutaneous transhepatic biliary drainage (PTBD);however, recently developed endoscopic ultrasound-assisted methods are gainingmore atention. Selecting the best available method in any specific scenario iscrucial, yet sometimes challenging. Thus, this review aimed to discuss theavailable techniques, indications, perks, pitfalls, and timing-related issues in themanagement of hCCA. In a preoperative setting, PTBD appears to have someadvantages: low risk of postprocedural complications (namely cholangitis) andbetter priming for surgery. For palliative purposes, we propose ERCP/PTBDdepending on the experience of the operators, but also on other factors: the levelof bilirubin (if very high, rather PTBD), length of the stenosis and the presence ofcholangitis (PTBD), ERCP failure, or altered biliary anatomy.

Preoperative evaluation with T-staging system for hilar cholangiocarcinoma

AIM： To investigate the clinical value of T-staging system in the preoperative assessment of hilar cholangiocarcinoma. METHODS： From March 1993 to January 2006, 85 patients who had cholangiocarcinoma diagnosed by operative tissue-biopsy were placed into one of three stages based on the new T-staging system, and it was evaluated the resectability and survival correlated with T-staging. RESULTS： The likelihood of resection and achieving tumor-free margin decreased progressively with increasing T stage （P 〈 0.05）. The cumulative 1-year survival rates of T1, T2 and T3 patients were 71.8%, 50.8% and 12.9% respectively, and the cumulative 3-year survival rate was 34.4%, 18.2% and 0% respectively; the survival of different stage patients differed markedly （P 〈 0.001）. Median survival in the hepatic resection group was greater than in the group that did not undergo hepatic resection （28 mo vs 18 mo; P 〈 0.05）. The overall accuracy for combined MRCP and color Doppler Ultrasonagraphy detecting disease was higher than that of combined using CT and color Doppler Ultrasonagraphy （91.4% vs 68%; P 〈 0.05 ）. And it was also higher in detecting port vein involvement （90% vs 54.5%; P 〈 0.05）.CONCLUSION： The proposed staging system for hilar cholangiocarcinoma can accurately predict resectability, the likelihood of metastatic disease, and survival. A concomitant partial hepatectomy would help to attain curative resection and the possibility of longterm survival. MRCP/MRA coupled with color Doppler UItrasonagraphy was necessary for preoperative evaluation of hilar cholangiocarcinoma.（OR = 2.46, 95% CI = 0.98-6.14）, and a significantly elevated risk of developing esophageal cancer among alcohol drinkers among alcohol drinkers （OR = 9.86, 95% CI = 3.10-31.38）. CONCLUSION： ADH2 and ALDH2 genotypes areassociated with esophageal cancer risk. ADH2＊1 allele and ALDH2＊2 allele carriers have a much higher risk of developing esophageal cancer, especially among alcohol drinkers.

聚焦胆管可切除性评估——医学影像技术和三维重建在肝门部胆管癌根治术术前规划中的应用

肝门部胆管癌是胆道系统的常见恶性肿瘤,根治性手术是重要的治疗手段之一。肝门部空间狭小、解剖变异率高,根治术难度大。应用医学影像技术和三维重建,术者可以在术前精准判断肝门部胆管癌的分期分型,参考胆管分离极限点(U点、P点)对肝门部胆管癌的可切除性进行评估,并预估门静脉、胆管、动脉变异对手术方案造成的影响,做好术前规划,提高根治性切除率,降低并发症发生率。

Diagnosis and treatment for advanced hilar cholangiocarcinoma:experience of 24 cases

The aim of this paper is to evaluate the effi-cacy of the surgical treatment for advanced hilar cholan-giocarcinoma(CCA)in order to improve the resection rate and curative effect.A retrospective analysis was performed on the data of 24 patients who had under-gone surgical treatment for advanced hilar CCA.According to the Bismuth classification,there were four cases of type IIIa,six cases of type IIIb,and 14 cases of type IV.Based on the treatment approaches,these patients were divided into three groups:①Radical resection group:There were five cases(one type IIIa,three type IIIb,and one type IV).The tumor visible to the naked eyes was resected thoroughly and the cut mar-gin was free of tumor by microscopic examination.Then,Roux-en-Y hepatico-jejunal anastomosis was per-formed to restore the bile flow.②Palliative resection group:There were 11 cases.The bile flow was restored by Roux-en-Y hepatico-jejunal anastomosis directly in five cases(two type IIIa,three type IIIb)and by internal drainage through a hepatico-jejunal bridge in the other six cases(one type IIIa,five type IV).③Simple internal biliary drainage group:There were eight cases of type IV,including three cases with the internal drainage through hepatico-jejunal bridge by laparotomy,three cases with endoscopic retrograde biliary drainage(ERBD),two cases with percutaneous transhepatic biliary drainage(PTBD).The rate of radical resection was 20.8%and the overall resection rate was 66.7%.All of the 24 patients were fol-lowed-up.The cumulative surviving rates were significantly different among these three groups(Log-rank x2=17.56,P=0.0002).For advanced hilar CCA,the best choice of treatment is radical resection.If radical resection is impractical,palliative resection combined with partial hepatectomy can significantly prolong the survival time.Internal drainage through a hepatico-jejunal bridge can enhance the surgical resection rate and decrease the occur-rence rate of postoperative biliary leakage.

肝门部胆管癌的外科治疗与预后

目的比较肝门部胆管癌各种外科治疗方法的疗效.方法对我院1991年1月～2002年12月间入院治疗的52例肝门部胆管癌患者临床资料进行回顾总结.结果全组病例治疗方法包括:保守治疗5例、切除术29例、姑息性胆道引流术(包括胆肠吻合术和外引流术)18例.非手术组的平均生存期比手术组差,引流组的平均生存期比切除组差.联合肝叶切除组与肿瘤局部切除组的平均生存期无统计学差异.切除手术和姑息性胆道引流手术的减黄效果没有显著性差异.在姑息性胆道引流术中,单纯内引流(胆肠吻合术)和内+外引流术相对于单独外引流来说,减黄效果较好.切除手术与引流术相比差别没有显著性差异.在胆道引流术中,胆肠吻合术的术后生活质量较外引流术的高.切除组中联合肝叶切除与肿瘤局部切除组的死亡率无统计学差异.切除组与引流组的手术并发症率无统计学差异.切除组中,联合肝叶切除组与肿瘤局部切除组的手术并发症率无统计学差异.结论肝门部胆管癌治疗方式中,以手术切除特别是根治性切除术的生存率为高,黄疸减退情况和术后生活质量亦均较其他治疗方式好.对于无法切除的病例,姑息性胆肠吻合术,或胆肠吻合术同时做胆管内插管引流术效果比单独采用外引流术好,应尽量避免单独采用外引流术治疗肝门部胆管癌.

肝门胆管癌的治疗进展

肝门部胆管癌因其发生部位特殊、呈浸润性生长及与肝门部血管关系密切等特点给手术切除造成极大的困难.长期以来,肝门部胆管癌被认为是无法手术根治性切除的癌肿.近20年来,随着影像学和手术技术的进步,使肝门部胆管癌的诊断和治疗取得重大进步,手术切除率逐步提高,生存率得到明显改善.但是对于是否应行扩大根治术、血管切除重建,以及放化疗等的疗效问题,依然是肝胆外科及肿瘤科医生所面临的严峻挑战.本文就肝门部胆管癌的治疗进展作一综述.

肝门胆管癌44例治疗分析

目的探讨肝门胆管癌的治疗。方法回顾性分析我院2000～2005年收治的44例肝门胆管癌病人的临床资料。结果本组手术治疗40例，手术切除率为42．5％（17／40），根治性切除8例（20％），姑息性切除9例（22．5％），单纯内引流、外引流各10例，3例仅行剖腹探查。4例病人合并肝叶切除，术后1例出现肝功能衰竭。未行肝叶切除病人无1例发生肝功能衰竭。结论Bismuth-Corlette分型可指导手术方式选择，但能否切除取决于病期。黄疸较深且拟行较大肝叶切除术时应行术前减黄，可减少术后肝功能衰竭的发生。

“桥式”内引流术在肝门胆管癌外科治疗中的应用

目的 探讨对无法切除的肝门胆管癌患者行“桥式”内引流术的减黄效果和术式优点。方法 对 1990年 1月～2 0 0 0年 10月的 30例无法手术切除的肝门胆管癌患者行“桥式”内引流术 ,根据术中 B超 (IOU S)的扫查结果确定内引流术式 ,并在 IOU S引导下选择扩张的肝内胆管。结果 “桥式”内引流术后 7d总胆红素、转氨酶、谷胺酰转肽酶和碱性磷酸酶均明显下降 ,与术前比较有显著性差异 (P<0 .0 1)。近期减黄效果明显 ,疗效优良率为 93.33% (2 8/ 30 )。结论 “桥式”内引流术减黄效果可靠 ,疗效满意 ,操作简便 ,并发症少 。

3期肝门胆管癌疗效分析——附35例报告

目的:探讨3期肝门胆管癌病人的治疗方法和效果。方法:回顾分析2002年1月-2008年12月我科收治的35例3期肝门胆管癌病人的临床资料。结果:35例3期肝门胆管癌(Ⅳ型),其中15例仅行外引流,20例行内引流,其3、12、24、36个月的生存率分别是90%、40%、9%、0%,95%、60%、20%、5%,内引流组生存率高于外引流组(χ2=7.45,p<0.05)。结论:内引流,尤其是"T"管长臂留于肠攀内,对3期肝门胆管癌病人生存率的提高大有益处。

肝门部胆管癌的外科治疗及预后分析(附61例报告)

目的探讨高位胆管癌的外科手术及影响预后的因素,以提高对高位胆管癌的认识。方法回顾性分析2002年1月至2007年12月61例高位胆管癌的外科治疗的临床资料。结果61例高位胆管癌按Bismuth-corlitte分型,Ⅰ型5例,Ⅱ型12例,Ⅲa型10例,Ⅲb型8例,Ⅳ型26例。根治切除31例,姑息手术13例,内引流13例,PTCD4例。根治切除组平均中位生存期29.3个月,其1,3,5年生存率分别为75%,39.3%,3.6%。姑息手术组平均中位生存期18.9个月,1,3,5年生存率分别为72.7%,9.1%,0%。内引流组平均中位生存期4.5个月,1,3,5年生存率分别为20%,0%,0%。根治手术组生存率高于姑息手术组(χ2=14.20,P=0.0002)。姑息手术组术后生存率高于内引流组(χ2=4.68,P=0.0305)。多元回归分析显示,切缘阳性,肿瘤分期,淋巴结转移是影响预后的独立因素。结论外科根治手术是治疗肝门部胆管癌唯一有效的手段。

经腹放置TFE胆道内支架治疗不能切除的肝门部胆管癌

目的 探讨手术中经腹腔放置四氟乙烯 (tetrafluoroethlene,TFE)胆道内支架对不能切除的肝门部胆管癌患者行姑息性减黄治疗的效果。方法 对 15例经手术探查证实无法切除的肝门部胆管癌患者 ,于手术中找到并切开梗阻以下的胆总管 ,向肝内胆管探查 ,开通被肿瘤阻塞的胆管 ,放置 TFE胆道内支架支撑肿瘤狭窄段胆管 ,做姑息减黄治疗。本组共放置Wilson- Cook公司的 ST- 2胆道内支架 2 0根。其中 5例于左、右肝管各放一根 ,引流全肝 ;7例仅放置 1根 ,引流半肝 (右肝管 6例 ,左肝管 1例 )。结果 所有患者术后恢复良好 ,黄疸消退顺利 ,生存质量好。术后无黄疸生存时间 3月～ 2年以上。没有发生严重并发症。结论 手术中经腹腔放置 TFE胆道内支架是对不能切除的肝门部胆管癌患者行姑息减黄治疗的有效方法。本手术操作简单 ,内引流效果确切 ,患者生存质量良好 ,值得推广应用。

64例肝门部胆管癌患者的诊治分析

目的探讨肝门部胆管癌的诊断方法和外科治疗。方法对2002年1月至2009年12月收治的64例肝门部胆管癌的临床资料进行回顾性分析。结果联合应用B超、磁共振胰胆管造影(MRCP)对肝门部胆管确诊率为100%;52例患者获随访,随访率81.3%;手术病死率3.1%;手术切除42例,其中根治性切除22例;内引流16例;外引流6例。根治性切除、姑息性切除、内引流和外引流组术后中位生存时间分别为30.4、15.6、12.9、3.8个月。联合肝叶切除、单纯肿瘤局部切除中位生存时间分别为28.5、16.3个月。结论联合运用影像学检查方法可提高肝门部胆管癌的诊断率。根治性切除及联合肝叶切除是影响肝门部胆管癌患者疗效的主要因素,对无法行根治性切除者以内引流为首选治疗方法 。

Ⅲ型肝门部胆管癌的外科治疗(附35例分析)

目的总结Ⅲ型肝门部胆管癌的手术经验。方法回顾性分析我院1999年1月至2006年12月,行手术切除的35例Ⅲ型肝门部胆管癌的临床资料。Ⅲa型16例,行肝门部胆管切除8例,行联合右半肝+右侧尾状叶切除7例,行联合右半肝+尾状叶切除、门静脉分叉部切除主干左支吻合1例。Ⅲb型19例,行肝门部胆管切除8例,行联合左半肝+左侧尾状叶切除9例,行联合左半肝+尾状叶切除、门静脉分叉部切除主干右支吻合1例,行联合左半肝+尾状叶切除、门静脉分叉部切除主干右支吻合、肝固有动脉分叉部切除主干右支吻合1例。结果本组32例获得随访,随访时间18～113个月。肝门部胆管切除病例术后病理根治性切除率为37.5%,联合肝叶切除病例术后病理根治性切除率73.7%,3例联合肝叶切除+血管切除病例均获术后病理根治性切除。肝门部胆管切除术后并发症发生率为31.3%,联合肝叶切除组术后并发症发生率为31.6%。3例联合肝叶切除+血管切除病例术后均无胆肠吻合口漏、肝断面坏死、胆漏等严重并发症。结论联合肝叶切除,必要时行受累分叉部血管切除重建,有益于提高Ⅲ型肝门部胆管癌的根治性切除率,且不增加术后并发症的发生率。

肝门部胆管癌综合治疗的进展与争议

肝门部胆管癌早期诊断困难,预后较差,临床治疗存在诸多争议。目前,外科治疗仍是该病最主要的治疗方法。术前精准评估和充分准备,以及术中安全、有效、规范切除肿瘤是治疗成功的关键,以化学治疗(化疗)为主的综合治疗提高了患者的生存期。未来以肿瘤学理念为指导,包括手术、化疗、靶向、免疫和生物治疗多种模式的综合治疗是肝门部胆管癌治疗的发展趋势。

晚期肝门胆管癌的经腹减黄治疗

目的 总结晚期肝门胆管癌减黄治疗的经验。方法 对 4 3例晚期肝门胆管癌施行不同的内引流术并进行分析及随访。结果 术后并发应激性溃疡出血 37例 ,无胆瘘及腹腔感染 ,无死亡病例。术后两周黄疸明显减轻 30例 ,完全消退 7例 ,随访 36例 ,术后生存期最短 4个月 ,最长 4 3个月 ,至今存活 6例 ,生存期分别达 2 1、16、11、9、8和 7个月。结论 术前影像检查为术式的选择提供直接的依据 ;术式选择视患者营养状况及梗阻部位和术中探查情况而定 ;置管架桥内引流术操作简单 ,肝断面空肠 Roux-

肝门部胆管癌外科手术治疗的进展

肝门部胆管癌是一种胆道常见恶性肿瘤,临床上出现症状较晚,早期诊断困难,而且肝门部胆管癌所处的解剖位置特殊及其向周围组织、血管、神经浸润的特点,外科根治性切除率低,因而预后较差。实施肝门部胆管癌的根治性切除能够有效地提高患者生存率,包括对于侵犯的血管或转移的淋巴结的扩大切除以及肝移植术的应用均能提高患者的生存率。因此,寻求胆管癌外科手术有效的新的治疗方法,对于提高胆管癌的临床治疗具有十分重要的意义。

肝门部胆管癌预后因素分析

目的探讨肝门部胆管癌术后的预后因素。方法回顾性分析65例肝门部胆管癌患者临床资料,分析影响肝门部胆管癌预后的因素。结果不同手术方式术后1 a生存率、2 a生存率及中位生存期比较差异有统计学意义(P<0.05)。单因素分析发现,手术方式、淋巴结转移情况、周围血管浸润情况、病理组织分化程度和肿瘤TNM分期是影响预后的因素;COX模型多因素分析结果显示,手术方式、淋巴结转移情况和肿瘤病理组织分化程度是影响预后的独立因素。结论根治性切除及彻底的淋巴结清扫是改善肝门部胆管癌预后的重要措施。