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.

Regional variation in routes to diagnosis of cholangiocarcinoma in England from 2006 to 2017

BACKGROUND Incidence of cholangiocarcinoma(CCA)is rising,with overall prognosis remaining very poor.Reasons for the high mortality of CCA include its late presentation in most patients,when curative options are no longer feasible,and poor response to systemic therapies for advanced disease.Late presentation presents a large barrier to improving outcomes and is often associated with diagnosis via mergency presentation(EP).Earlier diagnoses may be made by Two Week Wait(TWW)referrals through General practitioner(GP).We hypothesise that TWW referrals and EP routes to diagnosis differ across regions in England.AIM To investigate routes to diagnosis of CCA over time,regional variation and influencing factors.METHODS We linked patient records from the National Cancer Registration Dataset to Hospital Episode Statistics,Cancer Waiting Times and Cancer Screening Programme datasets to define routes to diagnosis and certain patient characteristics for patients diagnosed 2006-2017 in England.We used linear probability models to investigate geographic variation by assessing the proportions of patients diagnosed via TWW referral or EP across Cancer Alliances in England,adjusting for potential confounders.Correlation between the proportion of people diagnosed by TWW referral and EP was investigated with Spearman’s correlation coefficient.RESULTS Of 23632 patients diagnosed between 2006-2017 in England,the most common route to diagnosis was EP(49.6%).Non-TWW GP referrals accounted for 20.5%of diagnosis routes,13.8%were diagnosed by TWW referral,and the remainder 16.2%were diagnosed via an‘other’or Unknown route.The proportion diagnosed via a TWW referral doubled between 2006-2017 rising from 9.9%to 19.8%,conversely EP diagnosis route declined,falling from 51.3%to 46.0%.Statistically significant variation in both the TWW referral and EP proportions was found across Cancer Alliances.Age,presence of comorbidity and underlying liver disease were independently associated with both a lower proportion of patients diagnosed via TWW referral,and a higher proportion diagnosed by EP after adjusting for other potential confounders.CONCLUSION There is significant geographic and socio-demographic variation in routes to diagnosis of CCA in England.Knowledge sharing of best practice may improve diagnostic pathways and reduce unwarranted variation.

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.

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.

Intraoperative frozen section diagnosis of bile duct margin for extrahepatic cholangiocarcinoma

AIM To evaluate the usefulness of frozen section diagnosis(FSD) of bile duct margins during surgery for extrahepatic cholangiocarcinoma(CCA). METHODS We retrospectively analyzed 74 consecutive patients who underwent surgery for extrahepatic CCA from 2012 to 2017, during which FSD of bile duct margins was performed. They consisted of 40 distant and 34 perihilar CCAs(45 and 55 bile duct margins, respectively). The diagnosis was classified into three categories: negative, borderline(biliary intraepithelial neoplasia-1 and 2, and indefinite for neoplasia), or positive. FSD in the epithelial layer, subepithelial layer, and total layer was compared with corresponding permanent section diagnosis(PSD) postoperatively.Then, association between FSD and local recurrence was analyzed with special reference to borderline.RESULTS Analysis of 100 duct margins revealed that concordance rate between FSD and PSD was 68.0% in the total layer, 69.0% in the epithelial layer, and 98.0% in the subepithelial layer. The extent of remaining biliary epithelium was comparable between FSD and PSD, and more than half of the margins lost > 50% of the entire epithelium, suggesting low quality of the samples. In FSD, the rate of negative margins decreased and that of borderline and positive margins increased according to the extent of the remaining epithelium. Diagnostic discordance between FSD and PSD was observed in 31 epithelial layers and two subepithelial layers. Alteration from borderline to negative was the most frequent(20 of the 31 epithelial layers). Patients with positive margin in the total and epithelial layers by FSD demonstrated a significantly worse local recurrence-free survival(RFS) compared with patients with borderline and negative margins, which revealed comparable local RFS. Patients with borderline and negative margins in the epithelial layer by PSD also revealed comparable local RFS. These results suggested that epithelial borderline might be regarded substantially as negative. When classifying the status of the epithelial layer either as negative or positive, concordance rates between FSD and PSD in the total, epithelial, and subepithelial layers were 95.0%, 93.0%, and 98.0%, respectively.CONCLUSION During intraoperative assessment of bile duct margin, borderline in the epithelial layer can be substantially regarded as negative, under which condition FSD is comparable to PSD.

Advances in diagnosis,treatment and palliation of cholangiocarcinoma:1990-2009

Several advances in diagnosis,treatment and palliation of cholangiocarcinoma(CC)have occurred in the last decades.A multidisciplinary approach to this disease is therefore recommended.CC is a relatively rare tumor and the main risk factors are:chronic inflammation, genetic predisposition and congenital abnormalities of the biliary tree.While the incidence of intra-hepatic CC is increasing,the incidence of extra-hepatic CC is trending down.The only curative treatment for CC is surgical resection with negative margins.Liver transplantation has been proposed only for selected patients with hilar CC that cannot be resected who have no metastatic disease after a period of neoadjuvant chemo-radiation therapy.Magnetic resonance imaging/magnetic resonance cholangiopancreatography,positron emission tomography scan,endoscopic ultrasound and computed tomography scans are the most frequently used modalities for diagnosis and tumor staging.Adjuvant therapy,palliative chemotherapy and radiotherapy have been relatively ineffective for inoperable CC.For most of these patients biliary stenting provides effective palliation.Photodynamic therapy is an emerging palliative treatment that seems to provide pain relief,improve biliary patency and increase survival. The clinical utility of other emerging therapies such as transarterial chemoembolization,hepatic arterial chemoinfusion and high intensity intraductal ultrasound needs further study.

Review of endoscopic techniques in the diagnosis and management of cholangiocarcinoma

Cholangiocarcinoma is a rare malignancy of the biliary tract. Key factors in determining therapeutic options include knowledge of tumor extent, anatomy and obtaining tissue diagnosis. Endoscopically, there are three modalities available to make the diagnosis of cholangiocarcinoma. These include endoscopic retrograde cholangiopancreatography, endoscopic ultrasound with fine needle aspiration and cholangioscopy. Management of cholangiocarcinoma endoscopically is typically confined to stent placement for palliative purposes or as a bridge to surgery. In this article, we will review the endoscopic techniques available for the diagnosis and management of cholangiocarcinoma.

Diagnosis and initial management of cholangiocarcinoma with obstructive jaundice

Cholangiocarcinoma is the second most common primary hepatic cancer. Despite advances in diagnostic techniques during the past decade, cholangiocarcinoma is usually encountered at an advanced stage. In this review, we describe the classification, diagnosis, and initial management of cholangiocarcinoma with obstructive jaundice.

Liquid biopsy:Precise diagnosis and therapy for cholangiocarcinoma

The following letter to the editor highlights the review titled“Liquid biopsy in cholangiocarcinoma:Current status and future perspective”in World J Gastrointest Oncol 2021;13:332-350.It is necessary to realize individualized therapy to improve the clinical prognosis of patients with cholangiocarcinoma.

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.

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.

Diagnosis and Treatment of Cholangiocarcinoma: A Consensus from Surgical Specialists of China

Cholangiocarcinoma refers to malignant tumors that develop in epithelial lining of biliary system, and it is divided into two categories according to tumor location, intrahepatic cholangiocarcinoma （ICC） and extrahepatic cholangiocarcinoma （ECC）. ICC occurs from the epithelial cells of the intrahepatic bile duct, its branches and interlobular biliary tree; and ECC is divided into hilar cholangiocarcinoma and distal cholangiocarcinoma by the circumscription at the confluence of cystic duct and the common hepatic duct.

CLINICOPATHOLOGIC FEATURES AND DIAGNOSISOF COMBINED HEPATOCELLULAR ANDCHOLANGIOCARCINOMA

Hepatoma cases(N=130) were analyzed through histochemical and immunohistochemical staining. There were 99 cases of hepatocellular carcinoma(HCC), 15 cholangiocarcinoma(CC), and 16 combined HCC and CC (HCC+CC). The clinical features and the cases accompanied with hepatitis and/or liver cirrhosis in the non-tumor liver tissue of HCC+CC Group were between HCC GrouP and CC Group. Histologically, there were 4 cases with trabecular, 4 with pseudoglandula, 3 with solid type in HCCWC Group. In these 11 cases, the CC area was less than 10% of the neoplasm- The cases were classified as HCCWC type I. There was no obvious stroma fibrosis. The rest 5 cases of HCC+CC showed tubular carcinoma. The CC area took up over 10% of the tumor. These cases were designed as HCC+CC type II. There was significant fibrosis in the stroma so that its CC area is indistinguishable from that of CC cases. The CC area of all HCC+CC cases was positive to mucin and EMA staining, the same to that of CC cases. Near 70% of the HCC+CC cases had intracytoplasmic glycogen in the HCC area. The HCC area was mucin and EMA negative, similar to that of HCC cases- We also observed transition areas between HCC and CC in both of the type I and type II HCC+CC cases. The areas were mucin negative but EMA positive. We concluded that HCC+CC bad HCC and CC area. with the characteristics of HCC of CC respectively. Histochemical mucin staining and immunohistochemical EMA staining were valuable in detection and diagnosis of the HCC+CC.

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.

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.

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.

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.