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.

“Hepatic hilum area priority,liver posterior first”:An optimized strategy in laparoscopic resection for type Ⅲ-Ⅳ hilar cholangiocarcinoma

BACKGROUND In recent years,pure laparoscopic radical surgery for Bismuth-Corlette type Ⅲ and Ⅳ hilar cholangiocarcinoma(HCCA)has been preliminarily explored and applied,but the surgical strategy and safety are still worthy of further improvement and attention.AIM To summarize and share the application experience of the emerging strategy of“hepatic hilum area dissection priority,liver posterior separation first”in pure laparoscopic radical resection for patients with HCCA of Bismuth-Corlette types Ⅲ and IV.METHODS The clinical data and surgical videos of 6 patients with HCCA of Bismuth-Corlette types Ⅲ and Ⅳ who underwent pure laparoscopic radical resection in our department from December 2021 to December 2023 were retrospectively analyzed.RESULTS Among the 6 patients,4 were males and 2 were females.The average age was 62.2±11.0 years,and the median body mass index was 20.7(19.2-24.1)kg/m^(2).The preoperative median total bilirubin was 57.7(16.0-155.7)μmol/L.One patient had Bismuth-Corlette type Ⅲa,4 patients had Bismuth-Corlette type Ⅲb,and 1 patient had Bismuth-Corlette type Ⅳ.All patients successfully underwent pure laparoscopic radical resection following the strategy of“hepatic hilum area dissection priority,liver posterior separation first”.The operation time was 358.3±85.0 minutes,and the intraoperative blood loss volume was 195.0±108.4 mL.None of the patients received blood transfusions during the perioperative period.The median length of stay was 8.3(7.0-10.0)days.Mild bile leakage occurred in 2 patients,and all patients were discharged without serious surgery-related complications.CONCLUSION The emerging strategy of“hepatic hilum area dissection priority,liver posterior separation first”is safe and feasible in pure laparoscopic radical surgery for patients with HCCA of Bismuth-Corlette types Ⅲ and Ⅳ.This strategy is helpful for promoting the modularization and process of pure laparoscopic radical surgery for complicated HCCA,shortens the learning curve,and is worthy of further clinical application.

Conversion surgery after gemcitabine and cisplatin plus durvalumab for advanced intrahepatic cholangiocarcinoma:A case report

BACKGROUND The combination of immune checkpoint inhibitors and chemotherapy has shown promising results for the treatment of advanced biliary tract cancer(BTC).Based on the results of the TOPAZ-1 trial,a gemcitabine and cisplatin plus durvalumab(GCD)regimen was recently approved as first-line therapy for patients with advanced BTC.However,post-GCD conversion surgery has not been previously studied.Herein,we describe a case of advanced intrahepatic cholangiocarcinoma(ICC)successfully treated with radical surgery after GCD.CASE SUMMARY A 65-year-old female diagnosed with advanced ICC with periductal infiltration into the hepatic hilum underwent eight cycles of GCD,followed by durvalumab maintenance treatment,with mild adverse events.Partial response was obtained.Subsequently,a conversion surgery with extended left hepatectomy and bile duct resection was performed.The resection margins were negative,and the pathological diagnosis was compatible with small duct type ICC.The patient remained disease-free for 8 months without adjuvant chemotherapy.CONCLUSION We describe the case of a patient who received successful conversion surgery after GCD treatment for advanced ICC.

Compliance with enhanced recovery after surgery predicts long-term outcome after hepatectomy for cholangiocarcinoma

BACKGROUND Enhanced recovery after surgery(ERAS)program has been proved to improve postoperative outcome for many surgical procedures,including liver resection.There was limited evidence regarding the feasibility and benefit of ERAS in patients who underwent liver resection for cholangiocarcinoma.AIM To evaluate the feasibility of ERAS in patients who underwent liver resection for cholangiocarcinoma and its association with patient outcomes.METHODS We retrospectively analyzed 116 cholangiocarcinoma patients who underwent hepatectomy at Srinagarind Hospital,Khon Kaen University between January 2015 and December 2016.The primary outcome was the compliance with ERAS.To determine the association between ERAS compliance and patient outcomes.the patients were categorized into those adhering more than and equal to 50%(ERAS≥50),and below 50%(ERAS<50)of all components.Details on type of surgical procedure,preoperative and postoperative care,tumor location,postoperative laboratory results,and survival time were evaluated.The compliance with ERAS was measured by the percentage of ERAS items achieved.The Kaplan-Meier curve was used for survival analysis.RESULTS The median percentage of ERAS goals achieved was 40%(±12%).Fourteen patients(12.1%)were categorized into the ERAS≥50 group,and 102 patients were in the ERAS<50 group.Postoperative hospital stay was significantly shorter in the ERAS≥50 group[8.9 d,95%confidence interval(CI):7.3-10.4 d]than in the ERAS<50 group(13.7 d,95%CI:12.2-15.2 d)(P=0.0217).No hepatobiliary-related complications or in-hospital mortality occurred in the ERAS≥50 group.Overall survival was significantly higher in the ERAS≥50 group.The median survival of the patients in the ERAS<50 group was 1257 d(95%CI:853.2-1660.8 d),whereas that of the patients in the ERAS≥50 group was not reached.CONCLUSION Overall ERAS compliance for patients who underwent liver resection for cholangiocarcinoma is poor.Greater ERAS compliance could predict in-hospital,short-term,and long-term outcomes of the patients.

Accurate resection of hilar cholangiocarcinoma using eOrganmap 3D reconstruction and full quantization technique

BACKGROUND For treatment of hilar cholangiocarcinoma(HCCA),the rate of radical resection is low and prognosis is poor,and preoperative evaluation is not sufficiently accurate.3D visualization has the advantage of giving a stereoscopic view,which makes accurate resection of HCCA possible.AIM To establish precise resection of HCCA based on eOrganmap 3D reconstruction and full quantification technology.METHODS We retrospectively analyzed the clinical data of 73 patients who underwent HCCA surgery.All patients were assigned to two groups.The traditional group received traditional 2D imaging planning before surgery(n=35).The eOrganmap group underwent 3D reconstruction and full quantitative technical planning before surgery(n=38).The preoperative evaluation,anatomical classification of hilar hepatic vessels,indicators associated with surgery,postoperative complications,liver function,and stress response indexes were compared between the groups.RESULTS Compared with the traditional group,the amount of intraoperative blood loss in the eOrganmap group was lower,the operating time and postoperative intestinal ventilation time were shorter,and R0 resection rate and lymph node dissection number were higher(P<0.05).The total complication rate in the eOrganmap group was 21.05%compared with 25.71%in the traditional group(P>0.05).The levels of total bilirubin,Albumin(ALB),aspartate transaminase,and alanine transaminase in the eOrganmap group were significantly different from those in the traditional group(intergroup effect:F=450.400,79.120,95.730,and 13.240,respectively;all P<0.001).Total bilirubin,aspartate transaminase,and alanine transaminase in both groups showed a decreasing trend with time(time effect:F=30.270,17.340,and 13.380,respectively;all P<0.001).There was an interaction between patient group and time(interaction effect:F=3.072,2.965,and 2.703,respectively;P=0.0282,0.032,and 0.046,respectively);ALB levels in both groups tended to increase with time(time effect:F=22.490,P<0.001),and there was an interaction effect between groups and time(interaction effect:F=4.607,P=0.004).In the eOrganmap group,there was a high correlation between the actual volume of intraoperative liver specimen resection and the volume of preoperative virtual liver resection(t=0.916,P<0.001).CONCLUSION The establishment of accurate laparoscopic resection of hilar cholangiocarcinoma based on preoperative eOrganmap 3D reconstruction and full quantization technology can make laparoscopic resection of hilar cholangiocarcinoma more accurate and safe.

Precise mapping of hilar cholangiocarcinoma with a skip lesion by SpyGlass cholangioscopy:A case report

BACKGROUND Cholangiocarcinoma(CC)is a very aggressive cancer with a poor prognosis.As surgery is the only curative therapy,preoperative evaluation of the tumor extent is essential for surgical planning.Although high-quality image modalities such as computed tomography and magnetic resonance imaging have been used extensively in preoperative evaluation,the accuracy is low.To obtain precise localization of tumor spread arising from the hilar region preoperatively,the development of an acceptable imaging modality is still an unmet need.CASE SUMMARY A 52-year-old female presented to our emergency department with jaundice,abdominal pain,and fever.Initially,she was treated for cholangitis.Endoscopic retrograde cholangiopancreatography with the cholangiogram showed long segment filling defect in the common hepatic duct with dilatation of bilateral intrahepatic ducts.Transpapillary biopsy was performed,and the pathology suggested intraductal papillary neoplasm with high-grade dysplasia.After treatment of cholangitis,contrasted-enhanced computed tomography revealed a hilar lesion with undetermined Bismuth-Corlette classification.SpyGlass cholan gioscopy showed that the lesion involved the confluence of the common hepatic duct with one skip lesion in the posterior branch of the right intrahepatic duct,which was not detected by previous image modalities.The surgical plan was modified from extended left hepatectomy to extended right hepatectomy.The final diagnosis was hilar CC,pT2aN0M0.The patient has remained disease-free for more than 3 years.CONCLUSION SpyGlass cholangioscopy may have a role in precision localization of hilar CC to provide surgeons with more information before the operation.

Current interventional options for palliative care for patients with advanced-stage cholangiocarcinoma

Primary biliary tract tumors are malignancies that originate in the liver,bile ducts,or gallbladder.These tumors often present with jaundice of unknown etiology,leading to delayed diagnosis and advanced disease.Currently,several palliative treatment options are available for primary biliary tract tumors.They include percutaneous transhepatic biliary drainage(PTBD),biliary stenting,and surgical interventions such as biliary diversion.Systemic therapy is also commonly used for the palliative treatment of primary biliary tract tumors.It involves the administration of chemotherapy drugs,such as gemcitabine and cisplatin,which have shown promising results in improving overall survival in patients with advanced biliary tract tumors.PTBD is another palliative treatment option for patients with unresectable or inoperable malignant biliary obstruction.Biliary stenting can also be used as a palliative treatment option to alleviate symptoms in patients with unresectable or inoperable malignant biliary obstruction.Surgical interventions,such as biliary diversion,have traditionally been used as palliative options for primary biliary tract tumors.However,biliary diversion only provides temporary relief and does not remove the tumor.Primary biliary tract tumors often present in advanced stages,making palliative treatment the primary option for improving the quality of life of patients.

Proposal of a modified classification for hilar cholangiocarcinoma

Hilar cholangiocarcinoma is a malignant tumor that originates from the left and right hepatic ducts and their confluence.It is highly malignant and associated with a poor prognosis.Surgical resection is the only available curative treatment option.A scientific classification system can aid in the preoperative assessment of resectability and guide the development of appropriate surgical strategies.Several classification systems are available,with the Bismuth-Corlette(BC)classification being the earliest and most widely used.Similar to many other classifications,the BC classification relies on the secondary branching of the bile ducts as an important anatomical landmark,making it unsuitable for cases with variations in the bile duct anatomy.With advances in understanding the hepatic plate and anatomical structures at the hilum,the secondary bile ducts are no longer considered important anatomical landmarks.Therefore,modifications to the BC classification are needed to align with modern anatomical improvements and advancements in surgical techniques.Herein,we propose a modification to the BC classification.In this new system,the boundary of the hilar plate is considered as limit of the proximal ductal margin and used as an anatomical landmark,rather than the concept of“secondary bile ducts”in the BC classification.

Hilar Cholangiocarcinoma with Synchronous Metastases to Breast and Skeletal Muscle: A Case Report and Literature Review

Hilar cholangiocarcinoma has been reputed as a slow growth tumor in the past. Skeletal muscle is one of the most unusual sites of metastasis from any malignancy. We report herein a case of hilar cholangiocarcinoma with synchronous metastases to skeletal muscle and breast, and review the literature. The patient was a 48-year-old woman who presented with jaundice and weight loss. An inoperable hilar cholangiocarcinoma was diagnosed. She was treated with a combination of endoscopic plastic stent biloiary drainage and three-dimensional conformal radiotherapy （3DCRT）. Good response was achieved. Ten months later, she exhibited with a painful metastatic mass in the muscle rectus femoris of left thigh and a painless mass in the left breast. She underwent operation to relieve the pain, but died from liver failure after 8 months. The literature only offers isolated cases of cholangiocarcinoma with distant metastases, of which the common sites were cervical lymph node, bone, and portal venous system. Most patients were presented with multiple metastases with extensive local disease.

Evaluation of MRI in Diagnosing Hilar Cholangiocarcinoma

Objective： To investigate the clinical application of MRI in the diagnosis and preoperative evaluation of hilar cholangiocarcinoma （HC）. Methods： Thirty-four cases of HC were examined with 1.5T super-conduct MR unit. The plain MRI and MRCP were followed by dynamic Gd-DTPA （0.1 mmol/kg） contrast-enhanced scanning, which was repeated at 30 s intervals in the first 3 min with the patient being remained in the same position. Delayed scanning was done at 5, 8, 10, 12 and 15 min respectively. 3D- DCE-MRA was performed in arterial and portal venous phase with an additional bolus injection of 15-20 mL Gd-DTPA （0.15-0.20 mmol/kg）. The contrast time-signal curve of both the liver and the tumor was drawn from DCE imaging, and both arterial and portal venous phase imaging were reconstructed with MIP. MRI findings were compared with surgical findings and pathology. Results： Hilar lesion, intrahepatic ducts dilatation and disconnect of main hepatic duct were shown in all cases. Thirty-one cases had tumor-delayed enhancement. In 3D-DCE-MRA, spiral artery deformed was displayed in 6 cases, infiltration, encasement or occlusion of portal vein was displayed in 18 cases. The accuracy of assessing tumor unresectability and resectability by preoperative MRI was 95.0% （19/20） and 78.6% （11/14） respectively. The total accuracy rate of MRI assessing was 88.2% （30/34）. Conclusion： All-in-one MR, including plain MR, MRCP, MR DCE and 3D-DCE-MRA, can accurately show the lesions and involvement of neighbouring tissues. It was of great value in diagnosis and preoperative evaluation of HC.

Prognostic factors and long-term outcomes of hilar cholangiocarcinoma:A single-institution experience in China

AIM: To evaluate the prognostic factors of hilar cholangiocarcinoma in a large series of patients in a single institution.METHODS: Eight hundred and fourteen patients with a diagnosis of hilar cholangiocarcinoma that were evaluated and treated between 1990 and 2014, of which 381 patients underwent curative surgery, were included in this study. Potential factors associated with overall survival(OS) and disease-free survival(DFS) were evaluated by univariate and multivariate analyses.RESULTS: Curative surgery provided the best long-term survival with a median OS of 26.3 mo. The median DFS was 18.1 mo. Multivariate analysis showed that patients with tumor size > 3 cm [hazard ratio(HR) = 1.482, 95%CI: 1.127-1.949; P = 0.005], positive nodal disease(HR = 1.701, 95%CI: 1.346-2.149; P < 0.001), poor differentiation(HR = 2.535, 95%CI: 1.839-3.493; P < 0.001), vascular invasion(HR = 1.542, 95%CI: 1.082-2.197; P = 0.017), and positive margins(HR = 1.798, 95%CI: 1.314-2.461; P < 0.001) had poor OS outcome. The independent factors for DFS were positive nodal disease(HR = 3.383, 95%CI: 2.633-4.348; P < 0.001), poor differentiation(HR = 2.774, 95%CI: 2.012-3.823; P < 0.001), vascular invasion(HR = 2.136, 95%CI: 1.658-3.236; P < 0.001), and positive margins(HR = 1.835, 95%CI: 1.256-2.679; P < 0.001). Multiple logistic regression analysis showed that caudate lobectomy [odds ratio(OR) = 9.771, 95%CI: 4.672-20.433; P < 0.001], tumor diameter(OR = 3.772, 95%CI: 1.914-7.434; P < 0.001), surgical procedures(OR = 10.236, 95%CI: 4.738-22.116; P < 0.001), American Joint Committee On Cancer T stage(OR = 2.010, 95%CI: 1.043-3.870; P = 0.037), and vascular invasion(OR = 2.278, 95%CI: 0.997-5.207; P = 0.051) were independently associated with tumorfree margin, and surgical procedures could indirectly affect survival outcome by influencing the tumor resection margin. CONCLUSION: Tumor margin, tumor differentiation, vascular invasion, and lymph node status were independent factors for OS and DFS. Surgical procedures can indirectly affect survival outcome by influencing the tumor resection margin.

Risk factors and classifications of hilar cholangiocarcinoma

Cholangiocarcinoma is the second most common primary malignant tumor of the liver.Perihilar cholangiocarcinoma or Klatskin tumor represents more than 50% of all biliary tract cholangiocarcinomas.A wide range of risk factors have been identified among patients with Perihilar cholangiocarcinoma including advanced age,male gender,primary sclerosing cholangitis,choledochal cysts,cholelithiasis,cholecystitis,parasitic infection(Opisthorchis viverrini and Clonorchis sinensis),inflammatory bowel disease,alcoholic cirrhosis,nonalcoholic cirrhosis,chronic pancreatitis and metabolic syndrome.Various classifications have been used to describe the pathologic and radiologic appearance of cholangiocarcinoma.The three systems most commonly used to evaluate Perihilar cholangiocarcinoma are the Bismuth-Corlette(BC) system,the Memorial Sloan-Kettering Cancer Center and the TNM classification.The BC classification provides preoperative assessment of local spread.The Memorial Sloan-Kettering cancer center proposes a staging system according to three factors related to local tumor extent:the location and extent of bile duct involvement,the presence or absence of portal venous invasion,and the presence or absence of hepatic lobar atrophy.The TNM classification,besides the usual descriptors,tumor,node and metastases,provides additional information concerning the possibility for the residual tumor(R) and the histological grade(G).Recently,in 2011,a new consensus classification for the Perihilar cholangiocarcinoma had been published.The consensus was organised by the European Hepato-PancreatoBiliary Association which identified the need for a new staging system for this type of tumors.The classification includes information concerning biliary or vascular(portal or arterial) involvement,lymph node status or metastases,but also other essential aspects related to the surgical risk,such as remnant hepatic volume or the possibility of underlying disease.

Retrospective cohort study Lower incidence of complications in endoscopic nasobiliary drainage for hilar cholangiocarcinoma

AIM:To identify the most effective endoscopic biliary drainage technique for patients with hilar cholangiocarcinoma.METHODS:In total,118 patients with hilar cholangiocarcinoma underwent endoscopic management[endoscopic nasobiliary drainage(ENBD)or endoscopic biliary stenting]as a temporary drainage in our institution between 2009 and 2014.We retrospectively evaluated all complications from initial endoscopic drainage to surgery or palliative treatment.The risk factors for biliary reintervention,post-endoscopic retrograde cholangiopancreatography(post-ERCP)pancreatitis,and percutaneous transhepatic biliary drainage(PTBD)were also analyzed using patient-and procedure-related characteristics.The risk factors for bilateral drainage were examined in a subgroup analysis of patients who underwent initial unilateral drainage.RESULTS:In total,137 complications were observed in92(78%)patients.Biliary reintervention was required in 83(70%)patients.ENBD was significantly associated with a low risk of biliary reintervention[odds ratio(OR)=0.26,95%CI:0.08-0.76,P=0.012].Post-ERCP pancreatitis was observed in 19(16%)patients.An absence of endoscopic sphincterotomy was significantly associated with post-ERCP pancreatitis(OR=3.46,95%CI:1.19-10.87,P=0.023).PTBD was required in 16(14%)patients,and Bismuth type III or IV cholangiocarcinoma was a significant risk factor(OR=7.88,95%CI:1.33-155.0,P=0.010).Of 102 patients with initial unilateral drainage,49(48%)required bilateral drainage.Endoscopic sphincterotomy(OR=3.24,95%CI:1.27-8.78,P=0.004)and Bismuth II,III,or IV cholangiocarcinoma(OR=34.69,95%CI:4.88-736.7,P<0.001)were significant risk factors for bilateral drainage.CONCLUSION:The endoscopic management of hilar cholangiocarcinoma is challenging.ENBD should be selected as a temporary drainage method because of its low risk of complications.

Palliation:Hilar cholangiocarcinoma

Hilar cholangiocarcinomas are common tumors of the bile duct that are often unresectable at presentation. Palliation, therefore, remains the goal in the majority of these patients. Palliative treatment is particularly indicated in the presence of cholangitis and pruritus but is often also offered for high-grade jaundice and abdominal pain. Endoscopic drainage by placing stents at endoscopic retrograde cholangio-pancreatography(ERCP) is usually the preferred modality of palliation. However, for advanced disease, percutaneous stenting has been shown to be superior to endoscopic stenting. Endosonography-guided biliary drainage is emerging as an alternative technique, particularly when ERCP is not possible or fails. Metal stents are usually preferred over plastic stents, both for ERCP and for percutaneous bili-ary drainage. There is no consensus as to whether it is necessary to place multiple stents within advanced hi-lar blocks or whether unilateral stenting would suffice. However, recent data have suggested that, contrary to previous belief, it is useful to drain more than 50% of the liver volume for favorable long-term results. In the presence of cholangitis, it is beneficial to drain all of the obstructed biliary segments. Surgical bypass plays a limited role in palliation and is offered primarily as asegment Ⅲ bypass if, during a laparotomy for resec-tion, the tumor is found to be unresectable. Photody-namic therapy and, more recently, radiofrequency abla-tion have been used as adjuvant therapies to improve the results of biliary stenting. The exact technique to be used for palliation is guided by the extent of the bili-ary involvement(Bismuth class) and the availability of local expertise.

Laparoscopy in the management of hilar cholangiocarcinoma

The use of minimally invasive surgery has become widely accepted in many gastrointestinal fields,even in patients with malignancy.However,performing laparoscopic resection for the treatment of hilar cholangiocarcinoma is still not universally accepted as an alternative approach to open surgery,and only a limited number of such procedures have been reporteddue to the difficulty of performing oncologic resection and the lack of consensus regarding the adequacy of this approach.Laparoscopy was initially limited to staging,biopsy and palliation.Recent technological developments and improvements in endoscopic procedures have greatly expanded the applications of laparoscopic liver resection and lymphadenectomy,and some reports have described the use of laparoscopic or robot-assisted laparoscopic resection for hilar cholangiocarcinoma as being feasible and safe in highly selected cases,with the ability to obtain an adequate surgical margin.However,the benefits of major laparoscopic surgery have yet to be conclusively proven,and carefully selecting patients is essential for successfully performing this procedure.

Liver transplantation for hilar cholangiocarcinoma

The most appropriate treatment for Klatskin tumor(KT)with a curative intention is multimodal therapy based on achieving resection with tumour-free margins(R0resections)combined with other types of neoadjuvant or adjuvant treatment(the most important factor affecting KT survival is the possibility of R0 resections,achieving 5-year survival rate of 40%-50%).Thirty to forty percent of patients with KT are inoperable and present a 5-year survival rate of 0%.In irresectable non-disseminated KT patients,using liver transplantation without neoadjuvant treatment,the 5-year survival rate increase to 38%,reaching 50%survival in early stage.In selected cases,with liver transplantation and neoadjuvant treatment(chemotherapy and radiotherapy),the actuarial survival rate is 65%at 5 years and 59%at 10 years.In conclusion,correct staging,neoadjuvant treatment,living donor and priority on the liver transplant waiting list may lead to improved results.

Management of hilar cholangiocarcinoma in the North of England: Pathology, treatment, and outcome

AIM: To assess the management and outcome of hilar cholangiocarcinoma (Klatskin tumor) in a single tertiary referral center.METHODS: The notes of all patients with a diagnosis of hilar cholangiocarcinoma referred to our unit for over an 8-year period were identified and retrospectively reviewed. Presentation, management and outcome were assessed.RESULTS: Seventy-five patients were identified. The median age was 64 years (range 34-84 years). Male to female ratio was 1:1. Eighty-nine percent of patients presented with jaundice. Most patients referred were under Bismuth classification 3a, 3b or 4. Seventy patients required biliary drainage, 65 patients required 152percutaneous drainage procedures, and 25 had other complications. Forty-one patients had 51 endoscopic drainage procedures performed (15 failed). Of these,36 subsequently required percutaneous drainage. The median number of drainage procedures for all patients was three, 18 patients underwent resection (24%), nine had major complications and three died post-operatively.The 5-year survival rate was 4.2% for all patients, 21%for resected patients and 0% for those who did not undergo resection (P = 0.0021). The median number of admissions after diagnosis in resected patients was two and three in non-resected patients (P＜0.05).Twelve patients had external-beam radiotherapy, seven brachytherapy, and eight chemotherapy. There was no significant benefit in terms of survival (P = 0.46) or hospital admissions.CONCLUSION: Resection increases survival but carries the risk of significant morbidity and mortality.Percutaneous biliary drainage is almost always necessary and endoscopic drainage should be avoided if possible.

Preoperative assessment of hilar cholangiocarcinoma:combination of cholangiography and CT angiography

BACKGROUND: Hilar cholangiocarcinoma is one of the most difficult carcinomas to manage because of the location of the main tumor at the hepatic hilus and the complex anatomy of the biliary, arterial, and portal systems. To plan an operation, it is important to acquire accurate information about the relationship between hilar cholangiocarcinoma and adjacent vessels. This study aimed to evaluate the clinical value of cholangiography combined with spiral CT three-dimensional (3D) angiography for a preoperative assessment of hilar cholangiocarcinoma. METHODS: From March 2007 to August 2009, cholangiography was performed in 13 patients with hilar cholangiocarcinoma. Meanwhile, contrast-enhanced abdominal scanning was performed using 16-slice spiral CT, and the 3D images of the hepatic artery and portal vein were acquired. The level and range of invasion of the hepatic artery, the portal vein, and the bile duct, the preoperative Bismuth classification, and T-staging were recorded and compared with those after surgical exploration. RESULTS: The hepatic artery and portal vein were reconstructed successfully in all these patients. Percutaneous transhepatic cholangiography was performed in 9 patients, endoscopic retrograde cholangiopancreatography in 1, and magnetic resonance cholangiopancreatography in 3. The CT angiography records of invasion of the hepatic artery were consistent with the results of explorations in these patients. The data from 5 of the 13 patients were consistent with those on invasion of the portal vein. The results of the Bismuth classification and the T-staging system were consistent with those of surgical exploration in 12 of the 13 patients. Seven of 8 patients who were estimated to be suitable for operation based on images were curatively treated and 5 who were judged to be unsuitable for curative operation by cholangiography and CT angiography were confirmed intraoperatively and underwent palliative procedures. CONCLUSIONS: Cholangiography combined with multi-slice spiral 3D CT angiography can satisfactorily delineate the local invasion of hilar cholangiocarcinoma and accurately evaluate the resectability. This approach, therefore, contributes to the planning of safe operation. (Hepatobiliary Pancreat Dis Int 2010; 9: 186-191)

Pathological aspects of so called "hilar cholangiocarcinoma"

Cholangiocarcinoma(CC) arising from the large intrahepatic bile ducts and extrahepatic hilar bile ducts share clinicopathological features and have been called hilar and perihilar CC as a group.However,"hilar and perihilar CC" are also used to refer exclusively to the intrahepatic hilar type CC or,more commonly,the extrahepatic hilar CC.Grossly,a major distinction can be made between papillary and non-papillary tumors.Histologically,most hilar CCs are well to moderately differentiated conventional type(biliary) carcinomas.Immunohistochemically,CK7,CK20,CEA and MUC1 are normally expressed,being MUC2 positive in less than 50% of cases.Two main premalignant lesions are known:biliary intraepithelial neoplasia(BilIN) and intraductal papillary neoplasm of the biliary tract(IPNB).IPNB includes the lesions previously named biliary papillomatosis and papillary carcinoma.A series of 29 resected hilar CC from our archives is reviewed.Most(82.8%) were conventional type adenocarcinomas,mostly well to moderately differentiated,although with a broad morphological spectrum;three cases exhibited a poorly differentiated cell component resembling signet ring cells.IPNB was observed in 5(17.2%),four of them with an associated invasive carcinoma.A clear cell type carcinoma,an adenosquamous carcinoma and two gastric foveolar type carcinomas were observed.

Principles of surgical resection in hilar cholangiocarcinoma

The aim of this article is to describe the surgical techniques for the treatment of hilar cholangiocarcinoma(HC).Resection with microscopically negative margin(R0) is the only way to cure patients with HC.Today,resection of the caudate lobe and part of segment Ⅳ,combined with a right or left hepatectomy,bile duct resection,lymphadenectomy of the hepatic hilum and sometimes vascular resection,is the standard surgical procedure for HC.Intraoperative frozen-section examination of proximal and distal biliary margins is necessary to confirm the suitability of resection.Although lymphadenectomy probably has little direct effect on survival,inaccurate staging information may influence post resection treatment recommendations.Aggressive venous and arterial resections should be undertaken in selected cases to achieve a R0 resection.The concept of 'no-touch proposed' in 1999 by Neuhaus et al combine an extended right hepatectomy with systematic portal vein resection and caudate lobectomy avoiding hilar dissection and possible intraoperative microscopic dissemination of cancer cells.More recently minor liver resections have been proposed for treatment of HC.As the hilar bifurcation of the bile ducts is near to liver segments Ⅳ,Ⅴ and Ⅰ,adequate liver resection of these segments together with the bile ducts can result in cure.