Biliary tract cancers comprise a heterogeneous collection of malignancies usually described as cholangiocarcinoma of the intra- or extrahepatic bile duct, including perihilar cholangiocarcinoma and gallbladder cancer.

A review of pertinent parts of the ESSO core curriculum for the UEMS diploma targets (Fellowships exam, EBSQ), based on updated and available guidelines for diagnosis, surgical treatment and oncological management of cholangiocarcinoma.

Following the outline from the ESSO core curriculum we present the epidemiology and risk factors for cholangiocarcinoma, as well as the rationale for the current diagnosis, staging, (neo-)adjuvant treatment, surgical management, and short- and long-term outcomes. The available guidelines and consensus reports (i.e. NCCN, BGS and ESMO guidelines) are referred to. Recognition of biliary tract cancers as separate entities of the intrahepatic biliary ducts, the perihilar and distal bile duct as well as the gallbladder is important for proper management, as they each provide distinct clinical, molecular and treatment profiles to consider.

Core competencies in knowledge to the diagnosis, management and outcomes of biliary tract cancers are presented.

In the field of hilar cholangiocarcinoma (HCCA) treatment, the value of caudate lobe resection (CLR) has not been fully elucidated. Most scholars advocate that the caudate lobe should be routinely resected. To further investigate this issue, this study aims to evaluate the impact of CLR on surgical outcomes of HCCA patients who are judged to have no obvious tumor invasion in the caudate lobe. A retrospective analysis was performed on Bismuth type II, III, or IV HCCA patients who underwent radical resection between October 2005 and April 2023 at three Chinese medical centers. Patients were divided into the CLR group and the no caudate lobe resection (No-CLR) group according to whether CLR was performed or not. Baseline and tumor characteristics as well as perioperative outcomes were compared between the two groups using propensity score matching (PSM). A total of 397 HCCA patients underwent radical resection and there were 146 patients in each group after PSM. After PSM, the mortality was similar between the two groups. However, patients in the CLR group had a higher incidence of postoperative ascites (43.8% vs 30.1%, P=0.021), liver failure (15.8% vs 6.2%, P=0.014) and intra-abdominal infection (19.2% vs 8.2%, P=0.010). The R0 rate in the CLR group was significantly higher than that in the No-CLR group (88.4% vs 76.0%, P=0.009). Nevertheless, patients undergoing CLR did not show any improvement in overall survival (OS) or recurrence-free survival (RFS). Multivariate analysis showed that CLR was not associated with improved long-term surgical outcomes. The high level of CA19-9 and lower tumor differentiation were associated with worse OS, and adjuvant therapy can significantly improve OS. Lower tumor differentiation and N2 were associated with worse RFS. In summary, there is not yet sufficient evidence to support the routine resection of the caudate lobe during surgery for HCCA. For patients without obvious tumor invasion in the caudate lobe, resection of the lobe should be carefully weighed for its benefits and risks.

Biliary tract carcinoma (BTC) is relatively rare and comprises a spectrum of invasive tumors arising from the biliary tree. The prognosis is extremely poor. The incidence of BTC is relatively high in Asian countries, and a high number of cases are diagnosed annually in China owing to the large population. Therefore, it is necessary to clarify the epidemiology and high-risk factors for BTC in China. The signs associated with BTC are complex, often require collaborative treatment from surgeons, endoscopists, oncologists, and radiation therapists. Thus, it is necessary to develop a comprehensive Chinese guideline for BTC.

This clinical practice guideline (CPG) was developed following the process recommended by the World Health Organization. The Grading of Recommendations Assessment, Development, and Evaluation approach was used to assess the certainty of evidence and make recommendations. The full CPG report was reviewed by external guideline methodologists and clinicians with no direct involvement in the development of this CPG. Two guideline reporting checklists have been adhered to: Appraisal of Guidelines for Research and Evaluation (AGREE) and Reporting Items for practice Guidelines in Healthcare (RIGHT).

The guideline development group, which comprised 85 multidisciplinary clinical experts across China. After a controversies conference, 17 clinical questions concerning the prevention, diagnosis, and treatment of BTC were proposed. Additionally, detailed descriptions of the surgical principles, perioperative management, chemotherapy, immunotherapy, targeted therapy, radiotherapy, and endoscopic management were proposed.

The guideline development group created a comprehensive Chinese guideline for the diagnosis and treatment of BTC, covering various aspects of epidemiology, diagnosis, and treatment. The 17 clinical questions have important reference value for the management of BTC.

Reported incidence of extrahepatic bile duct cancer is higher in Asians than in Western populations. Korea, in particular, is one of the countries with the highest incidence rates of extrahepatic bile duct cancer in the world. Although research and innovative therapeutic modalities for extrahepatic bile duct cancer are emerging, clinical guidelines are currently unavailable in Korea. The Korean Society of Hepato-Biliary-Pancreatic Surgery in collaboration with related societies (Korean Pancreatic and Biliary Surgery Society, Korean Society of Abdominal Radiology, Korean Society of Medical Oncology, Korean Society of Radiation Oncology, Korean Society of Pathologists, and Korean Society of Nuclear Medicine) decided to establish clinical guideline for extrahepatic bile duct cancer in June 2021.

Contents of the guidelines were developed through subgroup meetings for each key question and a preliminary draft was finalized through a Clinical Guidelines Committee workshop.

In November 2021, the finalized draft was presented for public scrutiny during a formal hearing.

The extrahepatic guideline committee believed that this guideline could be helpful in the treatment of patients.

Hilar cholangiocarcinoma (HCCA) is widely considered to have a poor prognosis. In particular, combined caudate lobe resection (CLR) as a strategy for radical resection in HCCA is important for improving the R0 resection rate. However, the criteria for R0 resection, necessity of CLR, optimal extent of hepatic resection, and surgical approach are still controversial. This review aimed to summarize the findings and discuss the controversies surrounding CLR. Numerous clinical studies have shown that combined CLR treatment for HCCA improves the R0 resection rate and postoperative survival time. Whether surgery for Bismuth type I or II is combined with CLR depends on the pathological type. Considering the anatomical factors, total rather than partial CLR is recommended to achieve a higher R0 resection rate. In the resection of HCCA, a proximal ductal margin greater than or equal to 10 mm should be achieved to obtain a survival benefit. Although there is no obvious boundary between the right side (especially the paracaval portion) and the right posterior lobe of the liver, Peng's resection line can serve as a reference marker for right-sided resection. Laparoscopic resection of the caudate lobe may be safer, more convenient, accurate, and minimally invasive than open surgery, but it needs to be completed by experienced laparoscopic doctors.

Most of the patients with gallbladder cancer (GBC), intrahepatic cholangiocarcinoma (iCCA), and peri-hilar cholangiocarcinoma (pCCA) present with advanced disease. Complete staging with multiphasic liver imaging is essential to determine the extent of disease. Operative goals should include a margin-negative resection, portal lymphadenectomy for staging, and sufficient remnant liver volume. Biliary tract malignancies have distinct mutational drivers (GBC and pCCA = ERBB2 in 20%; iCCA = fibroblast growth factor receptor 2 or isocitrate dehydrogenase 1 in 20%) amenable to therapy with inhibitors. Clinical trials assessing neoadjuvant, peri-operative, and adjuvant treatments continue to evolve and now include targeted inhibitors and the integration of hepatic arterial infusion.

Margin-negative (R0) resection is the strongest positive prognostic factor in perihilar cholangiocarcinoma (PHC). Due to its anatomic location, the caudate lobe is frequently involved in PHC. This review aimed to examine the impact of caudate lobe resection (CLR) in addition to hepatectomy and bile duct resection for patients with PHC.

The MEDLINE, EMBASE, and Cochrane databases were systematically reviewed from inception to October 2021 to identify studies comparing patients undergoing surgical resection with hepatectomy and bile duct resection with or without CLR for treatment of PHC. Outcomes included the proportion of patients achieving R0 resection, overall survival (OS), and perioperative morbidity.

Altogether, 949 studies were screened. The review included eight observational studies reporting on 1137 patients. The patients who underwent CLR had a higher likelihood of R0 resection (odds ratio [OR], 5.85; 95% confidence interval [CI], 2.64-12.95) and a better OS (hazard ratio [HR], 0.65; 95% CI, 0.54-0.79) than those who did not. The use of CLR did not increase the risk of perioperative morbidity (OR, 1.03; 95% CI, 0.65-1.63).

Given the higher likelihood of R0 resection, improved OS, and no apparent increase in perioperative morbidity, this review supports routine caudate lobectomy in the surgical management of PHC. These results should be interpreted with caution given the lack of high-quality prospective data and the high probability of selection bias.

Delayed recovery (DR) is very commonly seen in the patients undergoing laparoscopic radical biliary surgery, we aimed to investigate the potential risk factors of DR in the patients undergoing radical biliary surgery, to provide evidences into the management of DR.Patients who underwent radical biliary surgery from January 1, 2018 to August 31, 2020 were identified. The clinical characteristics and treatment details of DR and no-DR patients were compared and analyzed. Multivariable logistic regression analyses were conducted to identify the potential influencing factors for DR in patients with laparoscopic radical biliary surgery.We included a total of 168 patients with laparoscopic radical biliary surgery, the incidence of postoperative DR was 25%. There were significant differences on the duration of surgery, duration of anesthesia, and use of intraoperative combined sevoflurane inhalation (all P < .05), and there were not significant differences on American Society of Anesthesiologists, New York Heart Association, tumor-lymph node- metastasis, and estimated blood loss between DR group and control group (all P > .05). Multivariable logistic regression analyses indicated that age ≥70 years (odd ratio [OR] 1.454, 95% confidence interval [CI] 1.146-1.904), body mass index ≥25 kg/m2 (OR 1.303, 95% CI 1.102-1.912), alcohol drinking (OR 2.041, 95% CI 1.336-3.085), smoking (OR 1.128, 95% CI 1.007-2.261), duration of surgery ≥220 minutes (OR 1.239, 95% CI 1.039-1.735), duration of anesthesia ≥230 minutes (OR 1.223, 95% CI 1.013-1.926), intraoperative combined sevoflurane inhalation (OR 1.207, 95% CI 1.008-1.764) were the independent risk factors for DR in patients with radical biliary surgery (all P < .05).It is clinically necessary to take early countermeasures against various risk factors to reduce the occurrence of DR, and to improve the prognosis of patients.

Cholangiocarcinoma accounts for approximately 10% of all hepatobiliary tumors and represents 3% of all new-diagnosed malignancies worldwide. Intrahepatic cholangiocarcinoma (i-CCA) accounts for 10% of all cases, perihilar (h-CCA) cholangiocarcinoma represents two-thirds of the cases, while distal cholangiocarcinoma accounts for the remaining quarter. Originally described by Klatskin in 1965, h-CCA represents one of the most challenging tumors for hepatobiliary surgeons, mainly because of the anatomical vascular relationships of the biliary confluence at the hepatic hilum. Surgery is the only curative option, with the goal of a radical, margin-negative (R0) tumor resection. Continuous efforts have been made by hepatobiliary surgeons in order to achieve R0 resections, leading to the progressive development of aggressive approaches that include extended hepatectomies, associating liver partition, and portal vein ligation for staged hepatectomy, pre-operative portal vein embolization, and vascular resections. i-CCA is an aggressive biliary cancer that arises from the biliary epithelium proximal to the second-degree bile ducts. The incidence of i-CCA is dramatically increasing worldwide, and surgical resection is the only potentially curative therapy. An aggressive surgical approach, including extended liver resection and vascular reconstruction, and a greater application of systemic therapy and locoregional treatments could lead to an increase in the resection rate and the overall survival in selected i-CCA patients. Improvements achieved over the last two decades and the encouraging results recently reported have led to liver transplantation now being considered an appropriate indication for CCA patients.

Infrahepatic inferior vena cava (IVC) clamping reduces central venous pressure. However, controversies remain regarding its impact on postoperative complications, particularly, the incidence of postoperative pulmonary embolism (PE). The aim of the study was to determine the impact of IVC clamping on the incidence of PE in patients undergoing hepatectomy.

A pooled analysis of five prospective trials on patients who underwent hepatic resection over a period of 10 years was performed. Patients with infrahepatic IVC clamping were compared to patients without infrahepatic IVC clamping. Outcomes were studied by univariate and multivariate analyses.

Of 505 included patients, 141 patients had IVC clamping and 364 patients served as control group. The rate of postoperative PE was comparable between groups (3% vs. 3%; P = 0.762), as were postoperative morbidity (P = 0.932), bile leakage (P = 0.272), posthepatectomy hemorrhage (P = 0.095), and posthepatectomy liver failure (P = 0.605), respectively. No clinicopathological and intraoperative risk factors were found to predict the onset of PE. Subgroup analyses of patients with major hepatectomy and vascular resections confirmed no adverse perioperative outcomes to be associated with IVC clamping.

Infrahepatic IVC clamping does not increase the incidence of postoperative PE.

We aim to draw a conclusion which type of hepatectomy could be the priority for hilar cholangiocarcinoma patients.

Surgery is established as only potentially curative treatment for hilar cholangiocarcinoma. However, whether hepatectomy should be preferred to the left-side hepatectomy, which includes left hemihepatectomy, extended left hemihepatectomy, and left trisectionectomy, or right-side hepatectomy, which represents right hemihepatectomy, extended right hemihepatectomy, and right trisectionectomy, is debated. In this meta-analysis, we evaluated and compared the efficacy and safety of left-side hepatectomy and right-side hepatectomy in patients with hilar cholangiocarcinoma.

We systematically retrieved the MEDLINE, PubMed, and Cochrane library and related bibliography up to February 2020. The primary outcome is overall survival, and the secondary outcome includes 1-, 3-, and 5-year survival rates, morbidity, mortality, R0 resection rate, and operation time. Based on heterogeneity, fixed-effects model or random-effects models were established through meta-analysis.

Eleven studies (11 cohort studies, totally 1031 patients) were involved in this study. The overall survival of patients who underwent left-side hepatectomy was comparable to that of patients who underwent right-side hepatectomy (hazard ratio, 1.27 [95% confidence interval, 0.98-1.63]). And there was no significant difference observed in 1-year (relative risk, 1.01 [95% CI, 0.89-1.15]), 3-year (relative risk, 0.94 [95% confidence interval, 0.80-1.11]), and 5-year survival (relative risk, 0.82 [95% confidence interval, 0.67-1.01]) rates between the left-side hepatectomy group and the right-side hepatectomy group. Comparing with the right-side hepatectomy cluster, the hilar cholangiocarcinoma patients in the left-side hepatectomy cluster presented better overall postoperative morbidity (relative risk, 0.82 [95% confidence interval, 0.71-0.96]) and major postoperative morbidity (relative risk, 0.73 [95% confidence interval, 0.56-0.95]). The post-hepatectomy liver failure rate (relative risk, 0.22 [95% confidence interval, 0.09-0.56]) and procedure-related mortality (relative risk, 0.41 [95% confidence interval, 0.23-0.70]) in the left-side hepatectomy group were better than those of the right-side hepatectomy group. Besides, the R0 resection rate was similar between the left-side hepatectomy group and the right-side hepatectomy group (relative risk, 0.95 [95% confidence interval, 0.87-1.03]). And the operation time for the left-side hepatectomy was significantly longer than that for the right-side hepatectomy (mean difference, 38.68 [95% confidence interval, 7.41-69.95]).

Through meta-analysis, we explored the comparable long-term outcomes and better short-term outcomes in the left-side hepatectomy group as is compared to the right-side hepatectomy group of hilar cholangiocarcinoma patients. In this study, the evidence obtained might indicate that the choice of left-side hepatectomy or right-side hepatectomy depends on the site of hilar cholangiocarcinoma in every patient.

The role of laparoscopic surgery (Lap) and robotic surgery (Rob) for radical resection of hilar cholangiocarcinoma (HC) is not clear. We summarized the safety and feasibility of Lap and Rob for HC.

A search of all HC studies in English published on PubMed up to April 2020 was conducted. References from retrieved articles were reviewed to broaden the search.

In total, 23 reports were enrolled: 15 involving Lap, seven using Rob and one study reporting a minimally invasive approach (Lap or Rob, not specified). A total of 205 cases of HC were documented (Lap/Rob/not specified, 99/101/5): 37 cases of Bismuth type-I (Lap/Rob, 17/20), 22 cases of Bismuth type-II (Lap/Rob, 15/7), 68 cases of type-III (Lap/Rob, 39/29) and 13 cases of type-IV (Lap/Rob, 9/4). The pooled prevalence of R0 resection was 80.1% (Lap/Rob, 85.9%/71.0%). The weighted mean for operative time, blood loss and post-operative hospital stay was 458.4 min (Lap/Rob, 423.3/660.8 min), 615.3 mL (Lap/Rob, 521.0/1188.5 mL) and 14.0 days (Lap/Rob, 14.0/13.7 days), respectively. The pooled prevalence of conversion to open surgery, post-operative complications, and perioperative mortality was 9.1% (Lap/Rob, 12.2%/3.8%), 47.2% (Lap/Rob, 38.4%/61.3%) and 3.0% (Lap/Rob, 4.0%/2.0%), respectively.

With innovations in technology and gradual accumulation of surgical experience, the feasibility and safety of performing Lap and Rob for HC will improve.