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A 75-year-old man was referred to our department because of an enlarging intrahepatic mass detected on magnetic resonance imaging (MRI) follow-up for another disease. MRI showed hypointensity on T1-weighted imaging and hyperintensity on T2-weighted imaging in liver segment 4. Abdominal plain computed tomography (CT) indicated a low-density lesion with an unclear boundary, measuring approximately 4 cm × 3 cm in liver segment 4. Dynamic CT showed early rim enhancement and gradual central enhancement. Contrast-enhanced CT also showed occlusion of the portal vein in segment 4. As the possibility of intrahepatic cholangiocarcinoma could not be excluded on imaging studies, we performed laparoscopic left medial sectionectomy. Histologically, the lesion showed diminished numbers of hepatocytes with increased collagen fibers compared with normal, with no patent portal vein. We considered this lesion a reactive lesion caused by collapse of the liver parenchyma owing to localized obstruction and loss of the portal vein. This lesion was pathologically diagnosed as portal biliopathy. We experienced an extremely rare case of intrahepatic mass-forming portal biliopathy that mimicked a hepatic tumor, which was diagnosed by laparoscopic resection. Portal biliopathy rarely forms intrahepatic mass lesions and must be distinguished from a malignant hepatic tumor., (© 2024. Japanese Society of Gastroenterology.)

Introduction: Intrahepatic cholangiocarcinoma is the second most common liver cancer. Desmoplastic stroma may be revealed as distinctive histopathologic findings favoring intrahepatic cholangiocarcinoma. Meanwhile, a range of imaging manifestations is often accompanied with rich desmoplastic stroma in intrahepatic cholangiocarcinoma, which can indicate large bile duct ICC, and a higher level of cancer-associated fibroblasts with poor prognosis and weak treatment response., Areas Covered: We provide a comprehensive review of current state-of-the-art and recent advances in the imaging evaluation for diagnosis, staging, prognosis and treatment response of intrahepatic cholangiocarcinoma. In addition, we discuss precursor lesions, cells of origin, molecular mutation, which would cause the different histological classification. Moreover, histological classification and tumor microenvironment, which are related to the proportion of desmoplastic stroma with many imaging manifestations, would be also discussed., Expert Opinion: The diagnosis, prognosis, treatment response of intrahepatic cholangiocarcinoma may be revealed as the presence and the proportion of desmoplastic stroma with a range of imaging manifestations. With the utility of radiomics and artificial intelligence, imaging is helpful for ICC evaluation. Multicentre, large-scale, prospective studies with external validation are in need to develop comprehensive prediction models based on clinical data, imaging findings, genetic parameters, molecular, metabolic, and immune biomarkers.

Objectives: To determine imaging hallmarks for distinguishing intrahepatic mass-forming biliary carcinomas (IMBCs) from hepatocellular carcinoma (HCC) and to validate their diagnostic ability using Bayesian statistics., Methods: Study 1 retrospectively identified clinical and imaging hallmarks that distinguish IMBCs (n = 41) from HCC (n = 247) using computed tomography (CT) and magnetic resonance imaging (MRI). Study 2 retrospectively assessed the diagnostic ability of these hallmarks to distinguish IMBCs (n = 37) from HCC (n = 111) using Bayesian statistics with images obtained from a different institution. We also assessed the diagnostic ability of the hallmarks in the patient subgroup with high diagnostic confidence (≥ 80% of post-test probability). Two radiologists independently evaluated the imaging findings in studies 1 and 2., Results: In study 1, arterial phase peritumoral parenchymal enhancement on CT/MRI, delayed enhancement on CT/MRI, diffusion-weighted imaging peripheral hyperintensity, and bile duct dilatation were hallmarks indicating IMBCs, whereas chronic liver disease, non-rim arterial phase hyperenhancement on CT/MRI, enhancing capsule on CT/MRI, and opposed-phase signal drop were hallmarks indicating HCC (p = 0.001-0.04). In study 2, Bayesian statistics-based post-test probability combining all hallmark features had a diagnostic accuracy of 89.2% (132/148) in distinguishing IMBCs from HCC for both readers. In the high diagnostic confidence subgroup (n = 120 and n = 124 for readers 1 and 2, respectively), the accuracy improved (95.0% (114/120) and 93.5% (116/124) for readers 1 and 2, respectively)., Conclusions: Combined interpretation of CT and MRI to identify hallmark features is useful in discriminating IMBCs from HCCs. High post-test probability by Bayesian statistics allows for a more reliable non-invasive diagnosis., Key Points: • Combined interpretation of CT and MRI to identify hallmark features was useful in discriminating intrahepatic mass-forming biliary carcinomas from hepatocellular carcinoma. • Bayesian method-based post-test probability combining all hallmark features determined in study 1 showed high (> 90%) sensitivity and specificity for distinguishing intrahepatic mass-forming biliary carcinomas from hepatocellular carcinoma. • If the post-test probability or the confidence was ≥ 80% when combining the imaging features of CT and MRI, the high specificity of > 95% was achieved without any loss of sensitivity to distinguish hepatocellular carcinoma from intrahepatic mass-forming biliary carcinomas.

Cholangiocarcinoma (CCA) is a serious health challenge with low survival prognosis. The liver fluke, Opisthorchis viverrini, plays a role in the aetiology of CCA, through hepatobiliary abnormalities: liver mass (LM), bile duct dilation, and periductal fibrosis (PDF). A population-based CCA screening program, the Cholangiocarcinoma Screening and Care Program, operates in Northeast Thailand. Hepatobiliary abnormalities were identified through ultrasonography. A multivariate zero-inflated, Poisson regression model measured associations between hepatobiliary abnormalities and covariates including age, sex, distance to water resource, and history of O. viverrini infection. Geographic distribution was described using Bayesian spatial analysis methods. Hepatobiliary abnormality prevalence was 38.7%; highest in males aged > 60 years (39.8%). PDF was most prevalent (20.1% of males). The Standardized Morbidity Ratio (SMR) for hepatobiliary abnormalities was highest in the lower and upper parts of the Northeast region. Hepatobiliary abnormalities specifically associated with CCA were also more common in males and those aged over 60 years and distributed along the Chi, Mun, and Songkram Rivers. Our findings demonstrated a high risk of hepatobiliary disorders in Northeast Thailand, likely associated with infection caused by O. viverrini. Screening for CCA and improvement of healthcare facilities to provide better treatment for CCA patients should be prioritized in these high-risk areas.

Purpose: Intraductal papillary neoplasm of the bile duct (IPNB) is a precursor to invasive carcinoma and is a distinct pathologic diagnosis. The purpose of this study was to evaluate imaging features of IPNB on cross-sectional imaging studies with histopathologic correlation., Materials and Methods: In this IRB approved, HIPAA compliant retrospective observational analysis of 23 pathology proven IPNB tumors 22 imaging studies were reviewed, 14 CT and 8 MRI scans. Features evaluated in consensus by two subspecialty-trained abdominal radiologists included: presence of specific lesion/mass within the bile duct, location within the biliary tree, size, morphology, enhancement characteristics, and bile duct caliber., Results: Majority of the subjects (16/18, 90%) had definite intraluminal mass, of which 7 (39%) had a polypoid mass with upstream diffuse biliary ductal dilation and 5 (28%) had a plaque-like mass with focal stricture and upstream biliary ductal dilatation. 6/18 (33%) subjects had low grade dysplasia, most commonly intestinal subtype, 7/18 (39%) subjects presented with invasive component, commonly pancreaticobiliary subtype, and 5/18 (28%) presented with high grade dysplasia., Conclusion: IPNB has increased predilection for extrahepatic bile ducts, commonly presenting as either an intraluminal polypoidal mass with associated upstream biliary ductal dilation or a focal plaque like mass with associated ductal stricture at the site of the tumor., Competing Interests: Declaration of competing interest Author declares that she has no conflict of interest., (Copyright © 2020 Elsevier Inc. All rights reserved.)

A 69-year-old man was referred to our hospital because of appetite loss. Imaging showed a nodular tumor in the perihilar bile duct and a second flat lesion in the distal bile duct. Right hepatopancreaticoduodenectomy was performed, and the histopathological findings demonstrated that the perihilar and distal lesions were moderately and poorly differentiated adenocarcinoma, respectively, and anatomically separated. Furthermore, the resected specimens showed no pancreaticobiliary maljunction. Histological and TP53 gene analyses in a rare case of synchronous double bile duct cancers suggest that there are various genetic pathways through which bile duct cancer develops, highlighting the complexity of its pathogenesis.

Background: IPNB is very rare disease and most previous studies on IPNB were case series with a small number due to low incidence. The aim of this study is to validate previously known clinicopathologic features of intraductal papillary neoplasm of bile duct (IPNB) based on the first largest multicenter cohort., Methods: Among 587 patients previously diagnosed with IPNB and similar diseases from each center in Korea, 387 were included in this study after central pathologic review. We also reviewed all preoperative image data., Results: Of 387 patients, 176 (45.5%) had invasive carcinoma and 21 (6.0%) lymph node metastasis. The 5-year overall survival was 80.9% for all patients, 88.8% for IPNB with mucosal dysplasia, and 70.5% for IPNB with invasive carcinoma. According to the "Jang & Kim's modified anatomical classification," 265 (68.5%) were intrahepatic, 103 (26.6%) extrahepatic, and 16 (4.1%) diffuse type. Multivariate analysis revealed that tumor invasiveness was a unique predictor for survival analysis. (p = 0.047 [hazard ratio = 2.116, 95% confidence interval 1.010-4.433])., Conclusions: This is the first Korean multicenter study on IPNB through central pathologic and radiologic review process. Although IPNB showed good long-term prognosis, relatively aggressive features were also found in invasive carcinoma and extrahepatic/diffuse type., (Copyright © 2019 International Hepato-Pancreato-Biliary Association Inc. Published by Elsevier Ltd. All rights reserved.)

Background/purpose: The enhancement pattern in the hepatic arterial phase (HAP) of dynamic computed tomography (CT) is reportedly a prognostic marker in patients with intrahepatic cholangiocarcinoma (IHCC). This study was performed to clarify the significance of central hypo-enhancement in the HAP in patients with mass-forming IHCC., Methods: Forty patients who had undergone initial surgical resection for mass-forming IHCC were enrolled. The dynamic CT was scanned 40 s after contrast agent injection as the HAP. A radiologist classified the patients into three groups based on the vascular pattern: the hyper-enhancement group (Hyper group), rim-enhancement group (Rim group), and hypo-enhancement group (Hypo group). The surgical specimens were immunohistochemically stained for hypoxia-inducible factor 1 (HIF-1). The correlation with clinicopathological findings and HIF-1 expression was investigated., Results: The Hyper, Rim, and Hypo groups comprised 8, 7, and 25 patients, respectively. There were no significant correlations between the groups and clinicopathological factors. Overall survival (OS) was significantly worse in the Hypo than in the Hyper group (p = 0.03). OS was also significantly worse in the Rim + Hypo group (i.e., hypo-enhancement in the central tumor) than in the Hyper group (p = 0.04). Furthermore, inclusion in the Rim + Hypo group was a prognostic factor for OS (hazard ratio 6.68). High HIF-1 expression in the central part of the tumor was correlated with central hypo-enhancement (Hyper group: 25% and Rim + Hypo group: 72%)., Conclusions: Central hypo-enhancement was a prognostic factor in patients with IHCC. The high malignant potential of tumors with central hypo-enhancement might be associated with HIF-1 upregulation.

Non-invasive diagnosis of hepatocellular carcinoma (HCC) in cirrhotic patients requires demonstration of wash-in and wash-out on contrast-enhanced imaging. Recent studies have reported misclassification of mass-forming intrahepatic cholangiocarcinoma (MFCCC) as HCC. We aimed to analyze the contrast enhancement patterns of MFCCC, focusing especially on lesions mimicking HCC. We retrospectively evaluated all consecutive patients with MFCCC who underwent surgery between 2007 and 2017. Patients with mixed HCC-MFCCC were excluded. Two expert radiologists reviewed preoperative CT and MRI. Full-nodule hyperenhancement in the arterial phase in conjunction with hypoenhancement in the portal/late phase was classified as an "HCC-like pattern". Imaging of MFCCCs with an HCC-like pattern was reviewed by an additional radiologist blinded to clinical data. Ninety-two patients were analyzed. All patients were investigated with multiphase CT and 85 with MRI. Twelve tumors (13%) showed full-nodule arterial hyperenhancement. Of these, four were hypoenhancing in the portal/late phase. Overall, 4/92 (4%) MFCCCs (4/45 in patients with cirrhosis/hepatitis, 9%) showed an HCC-like pattern accounting for misclassification as HCC on imaging review. HCC-like MFCCCs accounted for 9% of single tumors ≤ 50 mm. All HCC-like MFCCCs occurred in patients with cirrhosis or hepatitis, whereas only 47% of non-HCC-like MFCCCs did so (p = 0.053). After a median follow-up of 29 months, all patients with HCC-like MFCCCs are alive and disease free (median 64 months). In conclusion, MFCCC was misdiagnosed as typical HCC in 4% of all cases and in 9% of patients with single tumors ≤ 50 mm or with cirrhosis/hepatitis. The risk of misdiagnosis should be considered prior to treatment planning.

Objective: Cholangiocarcinoma (CCA) is the second most common primary hepatic malignancy after hepatocellular carcinoma. The current standard palliative treatment, chemotherapy regimen with gemcitabine and cisplatin, prolongs overall survival only of a few months. Established locoregional therapies are not a curative option or an alternative to surgery in the treatment of CCA. We report a case of a patient affected by a cholangiocellular carcinoma at hepatic hilum treated by Electrochemotherapy (ECT) at our oncologic center., Case Presentation: A 71 years old male affected by a CCA at hepatic hilum was treated with ECT according to ESOPE guidelines. No complications occurred during ECT procedure. The patient was discharged after 10 days. The functional MR evaluation at 2 and at 4 months post-treatment showed a significant response without significant post-treatment adverse events. The Computed tomography (CT) assessment after 18 months did not show progression of disease., Conclusions: ECT is safe and its use could be suggested as a palliative treatment of advanced neoplastic lesions in which radical surgical treatment is not possible.

Rationale and Objectives: The purpose of this study was to establish and validate radiomics signatures based on ultrasound (US) medicine images to assess the biological behaviors of intrahepatic cholangiocarcinoma (ICC) in a noninvasive manner., Materials and Methods: This study consisted of 128 ICC patients. We focused on evaluating six pathological features: microvascular invasion, perineural invasion, differentiation, Ki-67, vascular endothelial growth factor, and cytokeratin 7. Region of interest (ROI) of ICC was identified by manually plotting the tumor contour on the grayscale US image. We extracted radiomics features from medical US imaging. Then, dimensionality reduction methods and classifiers were used to develop radiomic signatures for evaluating six pathological features in ICC. Finally, independent validation datasets were used to assess the radiomic signatures performance., Results: We extracted 1076 quantitative characteristic parameters on the US medicine images. Based on extracted radiomics features, the best performing radiomic signatures for evaluating microvascular invasion features were produced by hypothetical test + support vector machine (SVM), perineural invasion subgroup were least absolute shrinkage and selection operator + principal component analysis + support vector machine, differentiation subgroup were hypothetical test + decision tree, Ki-67 subgroup were hypothetical test + logistic regression, vascular endothelial growth factor subgroup were hypothetical test + Gradient Boosting Decision Tree (GBDT), and cytokeratin 7 subgroup were hypothetical test + bagging, respectively., Conclusion: Through the high-throughput radiomics analysis based on US medicine images, we proposed radiomics signatures that have moderate efficiency in predicting the biological behaviors of ICC noninvasively., (Copyright © 2019 The Association of University Radiologists. Published by Elsevier Inc. All rights reserved.)

In 2010, we reported on a 72-year-old patient with a large, unresectable cholangiocarcinoma with intrahepatic metastases, which was treated by stereotactic radiofrequency ablation (SRFA) in three consecutive sessions. Within the last nine years, the same patient has received seven additional ablation sessions for a total of ten recurrent intrahepatic lesions. One year after the last SRFA, the patient's liver function is still within the physiological range, suggesting that this approach is not only sufficient for locally controlling tumor disease, but also for sparing healthy tissue. Moreover, periods of hospitalization were relatively short, while procedure-related pain was generally mild. In summary, SRFA has turned an aggressive disease with a devastating prognosis into a chronic condition while improving the patient's quality of life.

The imaging of focal liver lesions is a common task in daily radiological routine. The objectives of diagnostic imaging are, in addition to lesion detection, the characterization of the lesion as well as the follow-up assessment after surgical or local treatment or under systemic therapy. This article presents the typical morphologies observed in computed tomography and magnetic resonance imaging of hepatocellular carcinomas and intrahepatic cholangiocarcinomas as the most important representatives of primary malignant liver tumors and juxtaposes them with benign primary liver lesions such as adenoma and focal nodular hyperplasia (FNH). In addition, relevant technical aspects of imaging are briefly summarized. Finally, the main and additional criteria of the Liver Imaging Reporting and Data System (LI-RADS®) classification, which are becoming increasingly established clinically for the evaluation of liver lesions in the cirrhotic liver, are presented.

Hepatocellular carcinoma (HCC) can be diagnosed noninvasively with contrast-enhanced dynamic computed tomography, magnetic resonance imaging, or ultrasonography on the basis of its hallmark imaging features of arterial phase hyperenhancement and washout on portal or delayed phase images. However, approximately 40% of HCCs show atypical imaging features, posing a significant diagnostic challenge for radiologists. Another challenge for radiologists in clinical practice is the presentation of many HCC mimickers such as intrahepatic cholangiocarcinoma, combined HCC-cholangiocarcinoma, arterioportal shunt, and hemangioma in the cirrhotic liver. The differentiation of HCCs from these mimickers on preoperative imaging studies is of critical importance. Hence, we will review the typical and atypical imaging features of HCCs and the imaging features of its common mimickers. In addition, we will discuss how to solve these challenges in practice., Competing Interests: The authors have no potential conflicts of interest to disclose., (Copyright © 2019 The Korean Society of Radiology.)

This is a cross sectional study to establish that Magnetic resonance cholangiopancreatography (MRCP) could be accepted as the most effective imaging modality for the diagnosis of cholangiocarcinoma. Cholangiocarcinoma is the most common malignant tumor of the bile duct. It is much less common than hepatocellular carcinoma, accounting for 5-30% of all hepatic malignancies. Clinically suspected patients with obstructive jaundice due to cholangiocarcinoma were referred to the Department of Radiology and Imaging, Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh. The study was carried out in 60 patients, of which 38(63%) were male and 22(37%) were female. Out of 60 cases 56 cases were truly diagnosed by MRCP, 4 were normal, among these 1 case was diagnosed as cholangiocarcinoma as compared with histopathology. Forty four (44) patients (73.2%) of the patients were in sixth decade of life. The overall sensitivity and specificity were found to be 98.2% and 75%. The diagnostic accuracy was 96.7% & positive predictive value was about 98%.

Background and Objective: Cholangiocarcinoma (CCA) is an aggressive malignancy with high prevalence rate in Asia. The CCA premalignant lesions, including Biliary intraepithelial neoplasia (Bil-IN) and Intraductal papillary neoplasm of biliary tract (IPNB), share a common carcinogenesis; however, on imaging, patterns of presentation are different. Patterns and imaging characteristics on ultrasonography (US) and Magnetic resonance imaging (MRI) of both Bil-IN and IPNB are reported herein., Methods: In this retrospective study of imaging findings in premalignant CCA, pathology-proven cases of Bil-IN and IPNB at Chulabhorn Hospital were analyzed. Demographics, locations of lesions, imaging characteristics of both Bil-IN and IPNB were assessed, compared, and described., Results: Twenty-one premalignant lesions, 13 Bil-INs and 8 IPNBs, from 18 patients were included. Both Bil-IN and IPNB lesions were found more commonly at the right than left intrahepatic ducts (66.7% vs. 33.3%), and had more peripheral than central locations (85.7% vs. 14.3%). On US, Bil-IN commonly presented as focal bile duct dilatation (76.9%), whereas IPNB was more variable with hyperechoic nodules (37.5%), focal bile duct dilatation (37.5%), and diffuse bile duct dilatation with intraductal nodules (25%). On MRI, focal bile duct dilatation and nonfunctioning bile excretion are the most sensitive findings with sensitivities in the range of 84.6% to 100%. The presence of intraductal nodules and connection to the biliary system are findings that were significantly different between IPNB and Bil-IN, 62.5% versus 7.7% (p = 0.014) and 75% versus 15.4% (p = 0.018), respectively., Conclusions: Premalignant lesions of CCA, including Bil-IN and IPNB, have different imaging presentations. Knowledge of imaging presentations may improve early detection and increase confidence in diagnosis.

Background: The five-year survival rate for successful surgical treatment of cholangiocellular cancer is only 20-40%, and in the case of an unresectable tumor, the life expectancy does not usually exceed 6 months. Survival decreases with the presence of jaundice, due to the spread of the tumor process along the bile ducts, leading to their obstruction. We report outcomes of patients with nonresectable bile duct carcinoma complicated by obstructive jaundice treated with Photodynamic Therapy (PDT)., Methods: Combined diagnosis and treatment included percutaneous cholangiostomy, intraductal video fluorescence diagnostics, photodynamic therapy, and bile duct stenting. All patients were treated at the Sechenov University Oncology Center in Moscow. The results of treatment of 33 patients have been presented. The intraductal diagnosis of malignant bile duct lesions was performed after cholangiostomy using the endovideofluorescence module for minimally invasive surgery and endoscopy. With the use of this method, it is the first time in Russia that it has become possible to obtain a videofluorescent image of the tumor and to determine the high level of photosensitizer accumulation in all cholangiocarcinoma patients. The preparations Photolon, Radachlorin, and Photosens were employed as photosensitizers (PS). Intraductal photodynamic therapy was used to achieve the antitumor effect. Laser power density was about 200 mW/cm 2 ., Results: We present initial results, improved the diagnostic possibilities in this difficult localization of carcinoma, and demonstrated the feasibility of prolongation of life without significant deterioration of its quality. The average survival time in the treatment group is 9.5 months., Conclusion: The treatment of patients with nonresectable cholangiocarcinoma with Photodynamic Therapy should be an available option. In this context, the additional use of intraductal endovideofluorescence diagnostics is a highly specific technique that allows reliable detection of the photosensitizer accumulation predominantly by the tumor tissue and appears promising. As shown by our experience, flourescent localization followed by Photodynamic Therapy, enabled us to improve diagnostic techniques and treat the tumor with improved outcome., (Copyright © 2019 Elsevier B.V. All rights reserved.)

Biliary mucinous cystic neoplasm (BMCN) is a rare intrahepatic neoplasm comprising approximately 5% of cystic liver lesions. It can cause diagnostic dilemmas with most common differentials being complex hepatic cyst, hydatid cyst and intraductal papillary neoplasm of the bile duct. Affecting middle-aged female population, BMCN presents variedly ranging from vague abdominal symptoms to obstructive jaundice. Preoperative diagnosis is difficult. Preoperative CT scan with intravenous contrast and carbohydrate antigen 19.9 levels may give a clue towards the diagnosis. Intraoperative frozen section or cyst fluid aspiration cytology might help confirm the diagnosis. Fine needle aspirations should not be performed if BMCN is suspected, and intraoperative cyst spillage should be avoided to prevent tumour dissemination. We present here a case with a very atypical presentation of BMCN and review its present literature in brief., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Background & Aims: Imaging characteristics for discriminating the malignant potential of intraductal papillary neoplasm of the bile duct (IPNB) still remain unclear. This study aimed to define the magnetic resonance (MR) imaging findings that help to differentiate IPNB with an associated invasive carcinoma from IPNB with intraepithelial neoplasia and to investigate their significance with respect to long-term outcomes in patients with surgically resected IPNB., Methods: This retrospective study included 120 patients with surgically resected IPNB who underwent preoperative MR imaging with MR cholangiography before surgery from January 2008 and December 2017 in two tertiary referral centers. Clinical and MR imaging features of IPNB with intraepithelial neoplasia (n = 34) and IPNB with an associated invasive carcinoma (n = 86) were compared. Regarding significant features for discriminating IPNB with or without an associated invasive carcinoma, recurrence-free survival (RFS) rates were evaluated., Results: Significant MR imaging findings for differentiating IPNB with an associated invasive carcinoma from IPNB with intraepithelial neoplasia were intraductal visible mass, tumor size ≥2.5 cm, multiplicity of the tumor, bile duct wall thickening, and adjacent organ invasion (all p ≤0.002). The 1-, 3-, and 5-year RFS rates for surgically resected IPNB were 93.8%, 79.1%, and 70.0%, respectively. RFS rates were significantly lower in patients with each significant MR imaging finding of IPNB with an associated invasive carcinoma than in those without significant MR imaging findings (all p ≤0.039)., Conclusions: MR imaging with MR cholangiography may be helpful in differentiating IPNB with an associated invasive carcinoma from IPNB with intraepithelial neoplasia. Significant MR imaging findings of IPNB with an associated invasive carcinoma have a negative impact on RFS., Lay Summary: Significant magnetic resonance imaging findings that differentiated between an intraductal papillary neoplasm of the bile duct (IPNB) with an associated invasive carcinoma and an IPNB with intraepithelial neoplasia were intraductal visible mass, tumor size ≥2.5 cm, multiplicity of the tumor, bile duct wall thickening, and adjacent organ invasion. Significant magnetic resonance imaging findings of invasive IPNB have a negative impact on recurrence-free survival., (Copyright © 2018 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.)

Aims: Cholangiocarcinoma is a rare cancer arising from the biliary tree. Case series indicate that 25-40% of all borderline resectable primary tumours are potentially resectable. The Memorial Sloane Kettering System (MSKCC) stratifies patients for resectability by longitudinal and radial extension of the hilar tumour. The Bismuth-Corlette system describes the longitudinal extension of the tumour within the biliary duct system. We sought to validate and, if possible, augment these two scores within an independent validation cohort., Methods: Patients diagnosed with hilar cholangiocarcinoma between January 2009 and December 2016 were analysed from a prospectively held database. Patients with distal cholangiocarcinoma, peripheral cholangiocarcinoma and gallbladder cancer were excluded. Comparison of surgical findings to pre-operative radiological imaging was undertaken at the time of surgery., Results: The validation cohort was formed of 198 patients, of which, 55 (27.8%) patients underwent resection. Logistic regression analyses identified that BC score, MSKCC score, age at diagnosis and left artery involvement were all significant independent predictor's univariately. BC score explained 28% of the variability in resectability compared to 26% explained by MSKCC. In combination, the model consisting of BC score, age at diagnosis and left artery involvement explained 39% of variability in resectability compared to the 34% explained same model including MSKCC score instead of BC score., Conclusion: In this cohort an augmented BC score, incorporating left hepatic artery involvement, is more discriminative in predicting resectability than the current MSKCC system., (Copyright © 2018 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.)

Neuroendocrine tumors usually develop in the gastrointestinal tract, pancreas, and lung. Therefore, a neuroendocrine tumor of the bile duct is quite rare. We present a 59-year-old-male patient whose preoperative diagnosis was hilar cholangiocarcinoma. One month after embolization of the left branch and anterior branch of the portal vein, he underwent left hepatic trisegmentectomy and extrahepatic bile duct resection. Pathological examination revealed the neuroendocrine tumor in the submucosal layer of the hilar bile duct. Because there was no neuroendocrine tumor in other organs, the tumor was considered a primary neuroendocrine tumor of the hilar bile duct, rather than a liver metastasis from other organs. We also present a review of the English literature regarding neuroendocrine tumors of the bile duct., (Copyright© 2019, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Background: Hilar malignancy can occasionally be associated with high grade dysplasia (HGD) adjacent to invasive malignancy. For patients with HGD extending into the intrapancreatic bile duct, the authors adopted intrapancreatic bile duct resection (IP-BDR). The aims of this study were to compare the incidence of clinically relevant postoperative pancreatic fistula (CR-POPF), distal R0 resection and local recurrence within the distal bile duct remnant for patients undergoing extrahepatic bile duct resection without pancreaticoduodenectomy (with or without IP-BDR)., Methods: Patients who presented with hilar malignancy and underwent extrahepatic bile duct resection without pancreaticoduodenectomy between January 2005 and December 2016 were identified and the outcomes retrospectively evaluated., Results: Of 217 patients who met the inclusion criteria 62 (29%) patients underwent IP-BDR. There was a significant difference between patients undergoing standard resection vs. IP-BDR in terms of CR-POPF (5% (8/155) patients: vs 18% (11/62), p < 0.001). There were no significant differences between two groups of R0 status on distal margin (5% (8/155) patients: vs 10% (6/62), p = 0.359). No patient developed recurrence within the residual intrapancreatic bile duct., Discussion: The incidence of CR-POPF after IP-BDR for hilar malignancies was 18%. IP-BDR was associated with CR-POF, but does not appear to alter survival or local recurrence rate., (Copyright © 2018 International Hepato-Pancreato-Biliary Association Inc. Published by Elsevier Ltd. All rights reserved.)

Upper abdominal pain, icterus and cholestasis are the symptoms leading to evaluation of the biliary tract. Together with its complications biliary stone disease is the main reason for inflammation of the biliary system. A distinction is made between acute and chronic variants. In chronic bile duct inflammation primary sclerosing cholangitis (PSC), primary biliary cirrhosis (PBC) and more recently IgG4-associated sclerosing cholangitis are of particular importance. Besides benign and tumor-like-lesions, malignant entities as gallbladder carcinoma and cholangiocarcinoma (CCC) in its three locations have to be mentioned. Despite all recent improvements, specificity of bile-tract imaging still remains limited, especially regarding malignant masses. Therefore the final diagnosis is made in many cases by histological analysis.

A 68-year-old male who had undergone low anterior resection for primary rectal cancer 19 months ago presented with multiple CLM at Couinaud's segments IV, V, and VIII. There was no apparent macroscopic intrabiliary growth on preoperative computed tomography and gadolinium ethoxybenzyl diethylenetriamine pentaacetic acid-enhanced magnetic resonance imaging (EOB-MRI). However, the hepatobiliary phase of EOB-MRI revealed peritumoral low signal intensity in lesions in segments V and VIII, which indicates vascular invasion around hepatocellular carcinoma. Contrast-enhanced intraoperative ultrasound (CE-IOUS) clearly determined the extent of macroscopic glissonean growth from lesions in segments V and VIII, and more extensive resection was performed than was planned. Analysis of the resected specimens from segments V and VIII confirmed the presence of macroscopic intrabiliary growth with microscopic portal vein invasion. All three CLM were histopathologically diagnosed as well-to-moderately differentiated adenocarcinoma, and R0 resection was verified. Postoperative recovery was uneventful, and the patient was alive without evidence of recurrence 12 months after hepatic resection. CE-IOUS should be considered at the time of CLM resection, as it might enable more accurate detection of macroscopic intrabiliary growth of CLM, and enable resection with safer margins.

Background: We experienced a rare case of benign arterial stricture of the right posterior hepatic artery (RPHA) caused by atherosclerosis in a patient with hilar cholangiocarcinoma., Case Presentation: A 75-year-old man was referred to our hospital for the detailed investigation of serum hepatobiliary enzyme elevation. The patient had a history of hypertension, type 2 diabetes mellitus, and an operative history of coronary artery bypass grafting 10 years before. Endoscopic retrograde cholangiography found strictures of the right and left hepatic ducts with involvement of right anterior and posterior bile ducts. Adenocarcinoma was evident by brush cytology. We diagnosed these findings as hilar cholangiocarcinoma and planned left trisectionectomy including bile duct reconstruction. Although the tumor and RPHA were not adjacent, preoperative multidetector computed tomography revealed a stricture of the RPHA that was 5.6 mm in length. We suspected that atherosclerosis caused the stricture, and we performed digital subtraction angiography and intravascular ultrasonography that showed stricture of the RPHA accompanied by thick plaques in the arterial wall. We placed a bare-metal stent in the RPHA and then performed left trisectionectomy. Since this patient developed bile leakage postoperatively, percutaneous drainage was performed. The bile leakage was successfully controlled, and the patient was discharged 3 months after surgery. Unfortunately, 4 months after hepatectomy, he was re-hospitalized with multiple pyogenic liver abscesses. We performed intensive multimodal treatment for the liver abscesses and stabilized the disease; however, we eventually lost this patient due to liver failure 14 months after surgery., Conclusion: To the best of our knowledge, there is no previous literature on atherosclerosis of the RPHA, which was evident preoperatively in our case. Because arterial complications may lead to critical biliary complications in patients who undergo left trisectionectomy, we first performed prophylactic arterial stent placement. We speculate that existing chronic microscopic injury of the peribiliary plexus might have caused the liver abscesses. We successfully diagnosed atherosclerosis of the RPHA preoperatively. However, further investigation of patients is warranted to determine if left trisectionectomy is contraindicated in these patients.

Objective: We outline the concept of intraductal papillary neoplasm of the bile duct (IPNB), discuss the morphologic features of IPNB and the differential diagnoses, and describe the radiologic approaches used in multidisciplinary management., Conclusion: The concept of IPNB has been evolving. Because the imaging features of IPNB can be variable, different mimickers according to IPNB subtype can be considered. A multimodality approach is essential to obtain an optimal diagnosis and establish treatment plans.

Cholangiocarcinoma, the second most common hepatobiliary malignancy after hepatocellular carcinoma, is a heterogeneous disease entity with widely varying radiologic features, clinical behavior, and treatment approaches. In the detection, characterization, staging, and resectability assessment of cholangiocarcinoma, imaging studies are indispensable. Herein, an overview of the state-of-the-art imaging techniques is presented for the evaluation of intrahepatic and perihilar cholangiocarcinoma, as well as complementary multimodality and multiparametric imaging approaches for a more comprehensive evaluation. In addition, classification systems, new pathologic concepts in cholangiocarcinogenesis and premalignant lesions, and current trends in treatment approaches, which are vital to the imaging interpretation of cholangiocarcinoma, will be discussed. © RSNA, 2018.

Background/aim: Preoperative identification of the invasive component remains challenging in intraductal papillary neoplasm of the bile duct (IPNB). We evaluated the ability of preoperative 18 F-fluorodeoxyglucose positron emission tomography ( 18 F-FDG-PET) to differentiate between non-invasive IPNB, invasive IPNB, and papillary cholangiocarcinoma (CCA)., Patients and Methods: The maximum standardized uptake values (SUV max ) of 11 patients with IPNB (6 non-invasive and 5 invasive) and 20 with papillary CCA who underwent pre-surgical 18 F-FDG-PET were assessed. The SUV max cut-off that predicts an invasive component was determined using receiver operating characteristic (ROC) curve analysis., Results: The SUV max in patients with invasive IPNB and papillary CCA were significantly higher than in patients with non-invasive IPNB (p=0.035 and 0.0025, respectively). ROC curve analysis revealed an optimal SUV max cut-off of 4.5, which had 94.5% accuracy, 76.0% sensitivity, and 100% specificity., Conclusion: Our data suggest that the preoperative 18 F-FDG-PET SUV max can differentiate non-invasive IPNB from invasive IPNB and papillary CCA., (Copyright© 2018, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Objectives: To investigate the morphological classification of intraductal papillary neoplasm of the bile duct (IPNB), as well as morphological differences between IPNB without mucin secretion (IPNB-NM) and IPNB with mucin secretion (IPMN-B)., Methods: Eighty-one patients with IPNB were retrospectively analysed. Imaging examinations included computed tomography (CT), magnetic resonance imaging (MRI), gadolinium-ethoxybenzyl-diethylenetriaminepentaacetic acid (Gd-EOB-DTPA)-enhanced MRI and positron emission tomography-computed tomography (PET-CT). According to the morphology of tumours and extent of bile duct dilations, IPNB was classified into seven types: I, upstream-ductectatic type; II, typical type; III, superficial-spreading type; IV, no-mass-forming type; V, intrahepatic-cystic type; VI, extrahepatic-cystic type; and VII, infiltrating type., Results: Thirteen IPNB-NM patients comprised type I (11 cases), type II (1 case) and type VII (1 case); 68 IPMN-B patients comprised type I (2 cases), type II (30 cases), type III (6 cases), type IV (11 cases), type V (13 cases), type VI (2 cases) and type VII (4 cases). Bile duct dilations were more severe in IPMN-B than in IPNB-NM. PET-CT and Gd-EOB-DTPA-enhanced MRI clearly demonstrated the extension of infiltrating IPNB., Conclusions: IPNB can be classified into seven morphological types. IPNB-NM and IPMN-B have different morphological features., Key Points: • IPNB can be classified into seven morphological types. • IPNB-NM and IPMN-B have different morphological features. • Enhanced CT and MRI can display different types of IPNB. • Morphological classification of IPNB facilitates management of the disease.

Background: Patients with primary sclerosing cholangitis (PSC) have increased risk of cholangiocarcinoma (CCA). We evaluated pre-transplant work-up in PSC patients, to search for the most effective strategy for the detection of biliary dysplasia or early CCA., Methods: Two hundred and twenty five consecutive PSC patients undergoing liver transplantation (LTx) in Sweden between 1999 and 2013 were studied. Patients with CCA or dysplasia in the explanted liver were compared with those with benign histopathology. Measures of test performance were calculated for patients having brush cytology on one endoscopic retrograde cholangiopancreaticography (ERCP) occasion, for those having repeated examinations with or without cholangioscopy, and for fluorescence in situ hybridization (FISH). Survival after LTx was analyzed., Results: Brush cytology on a single ERCP occasion had moderate sensitivity (57%) and high specificity (94%) for the detection of CCA/high grade dysplasia (HGD) in the explanted liver. The corresponding sensitivity and specificity for FISH were 84% and 90%, respectively. Utilizing repeated ERCP and brush cytology to confirm the initial finding improved sensitivity to 82%. Using single operator cholangioscopy (SOC) for targeted examination at the second ERCP improved sensitivity (100%) and specificity (97%) significantly. Mortality rate in patients with incidentally discovered CCA (n = 16) in the explanted liver was significantly higher than in patients with HGD or benign histopathology (HR 16.0; 95% CI, 5.6-45.4; p < .001)., Conclusions: Repeated brush cytology especially when combined with targeted examination under SOC guidance is superior to single brush examinations. This strategy improves the detection of malignancy in PSC and is of importance for selection of patients for LTx.

Rationale and Objectives: This study aimed to investigate the potential of contrast-enhanced magnetic resonance imaging features to differentiate between mass-forming intrahepatic cholangiocellular carcinoma (ICC) and hepatocellular carcinoma (HCC) in cirrhotic livers., Materials and Methods: This study, performed between 2001 and 2013, included 64 baseline magnetic resonance imaging examinations with pathohistologically proven liver cirrhosis, presenting with either ICC (n = 32) or HCC (n = 32) tumors. To distinguish ICC form HCC tumors, 20 qualitative single-lesion descriptors were evaluated by two readers, in consensus, and statistically classified using the chi-square automatic interaction detection (CHAID) methodology. Diagnostic performance was assessed by a receiver operating characteristic analysis., Results: The CHAID algorithm identified three independent categorical lesion descriptors, including (1) liver capsular retraction; (2) progressive or persistent enhancement pattern or wash-out on the T1-weighted delayed phase; and (3) signal intensity appearance on T2-weighted images that could help to reliably differentiate ICC from HCC, which resulted in an AUC of 0.807, and a sensitivity and specificity of 68.8 and 90.6 (95% confidence interval 75.0-98.0), respectively., Conclusions: The proposed CHAID algorithm provides a simple and robust step-by-step classification tool for a reliable and solid differentiation between ICC and HCC tumors in cirrhotic livers., (Copyright © 2017 The Association of University Radiologists. Published by Elsevier Inc. All rights reserved.)

We report a case of a mucin-producing intraductal papillary neoplasm of the intrahepatic bile duct (M-IPNB) diagnosed over a period of 6 years. A 64-year-old man underwent follow-up evaluations for an abdominal aortic aneurysm at our hospital. In 2009, a computed tomography (CT) scan revealed a simple hepatic cyst in segment 3 of the liver. Annual CT scans initially showed almost no change in the size or shape of the cyst. The cystic lesion, which measured 5 cm in 2014, had increased to 11 cm by 2015, and a solid component was detected within the cyst. A biliary cystic tumor was suspected and we performed a left lateral hepatectomy. Pathological examination showed that the papillary lesion in the cyst included adenocarcinoma and adenoma components. We diagnosed M-IPNB in 2015. Identification of the solid component of the cyst, as well as an increase in cyst diameter in the image analyses, was critical for diagnosis of M-IPNB.

Background: Intrahepatic biliary cystadenomas are rare hepatic neoplasms, which are usually cystic. These tumors are often misdiagnosed as simple liver cysts and hydatid cysts clinically and radiologically owing to nonspecific clinical and radiologic features. These tumors require complete resection, as recurrence and malignant transformation can occur following incomplete excision. It is essential that these tumors be diagnosed accurately so that they can be adequately excised., Methods: Clinical and radiological features of six cases of biliary cystadenoma are described., Results: All of these cases were resected with the clinical and/or radiological impression of simple liver cysts and/or hydatid cysts. Out of the six patients, five were female and one was male. Ages of the patients ranged from 28 to 60 years (mean 45 years). The patients presented with nonspecific symptoms. Internal septations were seen on preoperative imaging (when available). On gross examination, all tumors were cystic; their sizes varied from 5.5 to 14 cm, mean size was 9.0 cm. On histopathologic examination, cystic spaces were lined by cuboidal to columnar mucin-secreting epithelium with underlying ovarian-type stroma. In one case, ovarian-type stroma was not seen. Recurrence was seen in three cases at 1 to 5 years of follow up., Conclusions: Owing to their malignant potential and high recurrence rate following incomplete resection, an aggressive surgical approach is recommended. Prognosis is excellent after complete resection.

Competing Interests: Competing interests: T. W. James has no competing interests. I. S. Grimm is a consultant for Boston Scientific. T. H. Baron has received travel and research support from Boston Scientific, ConMed, Cook Medical, Gore Medical, and Olympus, and is a consultant and speaker for Cook Endoscopy.

Objective: Intrahepatic cholangiocarcinoma (ICC) is a malignant tumor that arises from the intrahepatic bile ducts. Although the pathologic and imaging features of ICC have been clearly identified, recent updates have addressed the pathologic classification and imaging features of ICC using new imaging techniques. First, a proposed new pathologic ICC subclassification includes perihilar large duct and peripheral small duct ICCs. Second, advanced MR-based imaging features of ICC, such as hepatobiliary phase imaging using hepatocytespecific contrast material and DWI, have recently been described. These imaging features are important when differentiating ICCs from hepatocellular carcinomas. Finally, some imaging features of ICC, such as prominent arterial enhancement or degree of delayed enhancement, exhibit potential as prognostic imaging biomarkers., Conclusion: Comprehensive and updated knowledge of ICC is necessary for accurate diagnosis and could facilitate prediction of clinical outcomes for patients with ICC.

Objective: The aim of this project was to study the imaging characteristics of multi-detector CT (MDCT) in different types of malignant tumor in the common bile duct ampulla., Patients and Methods: We examined 30 cases of pancreatic head cancer, 35 of terminal cholangiocarcinoma, 26 of ampullary carcinoma, and 40 of benign lesions, all confirmed by pathology. We used 64-slice spiral CT plain scan and multi-phase enhanced scan with multi-planar reconstruction (MPR) and curved planar reconstruction (CPR) post-processing to obtain three-dimensional images. From these images, we analyzed intrahepatic and extrahepatic bile ducts, gallbladder and common bile duct dilation, and morphology and enhancement pattern of lesions and surrounding tissue., Results: The dilatation rate of intrahepatic, extrahepatic bile duct and gallbladder in terminal cholangiocarcinoma was the highest. The double duct sign was most evident in pancreatic head cancer. Ampullary carcinoma fell in between, and the benign lesions had no intrahepatic or extrahepatic bile duct and pancreatic duct dilation. Pancreatic cancer had a larger diameter, a higher internal rate of necrosis, and the surrounding tissues had a higher vulnerability to invasion. Terminal cholangiocarcinoma had a smaller diameter and a thicker wall. Benign lesions showed isodensity and hyperdensity shadow in the lumen, but no other significant changes were observed. Pancreatic head carcinoma had lower enhancement degree than normal pancreatic tissue, no enhancement in the internal necrotic area, and the borderline was unclear. Thickened ductal wall of the terminal cholangiocarcinoma showed equal density, enhancement and commonly delayed enhancement. The enhancement degree was higher than in the cancer of the pancreatic head and slightly lower than in ampullary cancer. Ampullary cancer had a regular margin and a significant enhancement, with enhancement degree higher than in pancreatic cancer and lower than in common bile duct cancer. Arterial and venous phases showed enhancement, but benign lesions did not show enhancement., Conclusions: MDCT imaging and post-processing techniques have significant application in the diagnosis of benign and malignant lesions, as well as a malignant tumor of the common bile duct ampulla.

Purpose: To investigate the use of dual source dual-energy CT (DECT) quantitative parameters compared with the use of conventional CT for differentiating small (≤3cm) intrahepatic mass-forming cholangiocarcinoma (IMCC) from small liver abscess (LA) during the portal venous phase (PVP)., Material and Methods: In this institutional review board-approved, retrospective study, 64 patients with IMCCs and 52 patients with LAs who were imaged in PVP using dual-energy mode were included retrospectively. A radiologist drew circular regions of interest in the lesion on the virtual monochromatic images (VMI), color-coded iodine overlay images, and linear blending images with a linear blending ratio of 0.3 to obtain CT value, its standard deviation, slope (k) of spectral curve and normalized iodine concentration (NIC). Two radiologists assessed lesion type on the basis of qualitative CT imaging features., Results: CT values on VMI at 50-130keV (20keV-interval), k, and NIC values were significantly higher in IMCCs than in LAs (p<0.0001). The best single parameter for differentiating IMCC from LA was CT value at 90keV, with sensitivity, specificity, accuracy, positive predictive value, and negative predictive value of 89.1%, 86.5%, 87.9%, 89.1%, and 86.5%, respectively. The best combination of parameters was CT value at 90keV, k, and NIC, with values of 87.5%, 84.6%, 83.6%, 87.5%, and 84.6%, respectively. Compared with CT value at linear blending images, CT value at 90keV showed greater sensitivity (89.1% vs 60.9%, p<0.0001) and similar specificity (86.5% vs 84.6%, p=1.0000), and combined CT value at 90keV, k, and NIC showed greater sensitivity (87.5% vs 60.9%, p<0.0001) and similar specificity (84.6% vs 84.6%, p=1.0000). Compared with qualitative analysis, CT value at 90keV showed greater sensitivity (89.1% vs 65.6%, p=0.0059) and specificity (86.5% vs 69.2%, p=0.0352), and combined CT value at 90keV, k, and NIC showed greater sensitivity (87.5% vs 65.6%, p=0.0094) and similar specificity (84.6% vs 69.2%, p >0.05)., Conclusion: Quantitative analysis of dual source dual-energy CT quantitative parameters showed greater accuracy than quantitative and qualitative analyses of conventional CT for differentiating small IMCCs from small LAs on single PVP scan., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Background: Since biliary variations are commonly seen, our aims are to clarify these insidious variations and discuss their surgicopathologic implications for Bismuth-Corlette (BC) type IV hilar cholangiocarcinoma (HC) applied to hemihepatectomy., Methods: Three-dimensional images of patients with distal bile duct obstruction (n = 97) and advanced HC (n = 79) were reconstructed and analyzed retrospectively. Normal biliary confluence pattern was defined as the peripheral segment IV duct (B4) joining the common trunk of segment II (B2) and segment III (B3) ducts to form the left hepatic duct (LHD) that then joined the right hepatic duct (RHD). The lengths from left and right secondary biliary ramifications to the right side of the umbilical portion of the left portal vein (Rl-L) and the cranio-ventral side of the right portal vein (Rr-R) were measured, respectively, and compared with the resectable bile duct length in HCs. Surgicopathologic findings were compared between different BC types., Results: The resectable bile duct length in right hemihepatectomy for eradication of type IV tumors was significantly longer than the Rl-L length in normal biliary configuration (17.4 ± 1.8 and 10.3 ± 3.4 mm, respectively, p < 0.001), and type III variation (B2 joining the common trunk of B3 and B4) was the predominant configuration (53.8%). The resectable length in left hemihepatectomy for eradication of type IV tumors was comparable with the Rr-R length in RHD absent cases (15.2 ± 2.5 and 16.4 ± 2.6 mm, respectively, p = 0.177) but significantly longer than that in normal configuration (p < 0.001). The estimated length was 8.5 ± 2.0 mm in unresectable cases. There was no significant difference between type III and IV tumors, except for the rate of nodal metastasis (29.7 and 76.0%, respectively, p < 0.001)., Conclusion: Hemihepatectomy might be selected for curative-intent resection of BC type IV tumors considering the advantageous biliary variations, whereas anatomical trisegmentectomy is recommended for the resectable bile duct length less than 10 mm. Biliary variations might result in excessive classification of BC type IV but require validation on further study.

An 82-year-old woman who had been suffering from repeated obstructive jaundice for 7 years was referred to our hospital. Although endoscopic aspiration of the mucin in the common bile duct had been temporally effective, origin of the mucin production had not been detectable. The patient thus had been forced to be on long-term follow-up without curative resection. Endoscopic retrograde cholangioscopy on admission revealed massive mucin in the common bile duct. In addition, an anomalous bile duct located proximal to the gallbladder was identified. Since the lumen of the anomalous duct was irregular and the rest of biliary tree was completely free of suspicious lesions, the anomalous duct was judged to be the primary site. Surgical resection of the segment 4 and 5 of the liver combined with the extrahepatic biliary tract was performed. Pathological diagnosis was compatible to intraductal papillary neoplasm with high-grade intraepithelial dysplasia of the anomalous bile duct. The patient has been free from the disease for 6.5 years after resection. This is the first case of intraductal papillary neoplasm derived from an anomalous bile duct, which was resected after long-term conservative treatment. The present case suggested the slow growing character of natural history of the neoplasm.

Purpose: The purpose of this study is to investigate the value of multidetector computed tomography (MDCT) assessment of resectability in hilar cholangiocarcinoma, and to identify the factors associated with unresectability and accurate evaluation of resectability., Methods: From January 2007 to June 2015, a total of 77 consecutive patients were included. All patients had preoperative MDCT (with MPR and MinIP) and surgical treatment, and were pathologically proven with hilar cholangiocarcinoma. The MDCT images were reviewed retrospectively by two senior radiologists and one hepatobiliary surgeon. The surgical findings and pathologic results were considered to be the gold standard. The Chi square test was used to identify factors associated with unresectability and accurate evaluation of resectability., Results: The sensitivity, specificity, and overall accuracy of MDCT assessment were 83.3 %, 75.9 %, and 80.5 %, respectively. The main causes of inaccuracy were incorrect evaluation of N2 lymph node metastasis (4/15) and distant metastasis (4/15). Bismuth type IV tumor, main or bilateral hepatic artery involvement, and main or bilateral portal vein involvement were highly associated with unresectability (P < 0.001). Patients without biliary drainage had higher accuracy of MDCT evaluation of resectability compared to those with biliary drainage (P < 0.001)., Conclusion: MDCT is reliable for preoperative assessment of resectability in hilar cholangiocarcinoma. Bismuth type IV tumor and main or bilateral vascular involvement highly suggest the unresectability of hilar cholangiocarcinoma. Patients without biliary drainage have a more accurate MDCT evaluation of resectability. We suggest MDCT should be performed before biliary drainage to achieve an accurate evaluation of resectability in hilar cholangiocarcinoma.

Background and Study Aims: The diagnosis of cholangiocarcinoma (CCA) is difficult. The present study aimed to assess the clinical features, diagnosis, and survival in CCA., Patients and Methods: This is a prospective study on 46 patients with CCA who underwent endoscopic retrograde cholangiopancreatography (ERCP) or surgical resection and 20 controls with a clinical and ERCP suspicion for CCA in whom surgical biopsy and/or 4-year follow-up showed a benign biliary stricture., Results: The median age at presentation was 71years (range 44-88). Thirty-four patients (73.9%) presented with painless jaundice. Median CA 19-9 value was 188IU/L (range 1-49,138), with a level of <100IU/L in 13 patients (28%). Total bilirubin was 11.9 (0.6-36.3)mg/dL. The tumour was intrahepatic in 3 (6.5%), hilar (Klatskin) in 25 (54.3%), and located in the lower third of the bile duct in 18 (39.1%) patients. The diagnosis was confirmed by positive cytology in 10 (21.7%), biopsy in 20 (43.5%), cholangioscopy in five (10.9%), and imaging and clinical grounds in 11 (23.9%) patients. Cytology was feasible in 36 patients; it was positive in 10 and "highly indicative" in two patients (33.3% sensitivity). Twenty-two patients (47.8%) were treated by surgical resection, and the rest were offered palliative biliary drainage. Mean estimated survival for the entire group of CCA patients was 21.5±3.3months. Survival was slightly longer in patients who underwent surgical resection than those who had palliative treatment; the estimated mean survival rates were 26.2±4.2 vs. 17.1±3.3months, respectively, but the difference was not statistically significant (p=0.115)., Conclusion: The diagnosis of CCA is difficult and often delayed. The outcome is generally poor., (Copyright © 2016 Pan-Arab Association of Gastroenterology. Published by Elsevier B.V. All rights reserved.)