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Objectives Adenocarcinomas of the biliary tract frequently present diagnostic challenges because of their histologic overlap with benign and preinvasive lesions. The molecular profiles of biliary adenocarcinomas vary by anatomical location. Variations in IDH1/2, common in intrahepatic cholangiocarcinoma, can lead to defective production of 5-hydroxymethylcytosine (5-hmC). Limited ancillary studies are available for biliary adenocarcinomas, and loss of 5-hmC staining could serve as a helpful ancillary diagnostic tool for biliary tract malignancies. Methods We evaluated 93 cases—20 benign biliary lesions, 15 preinvasive biliary neoplasms, 46 invasive biliary carcinomas, and 12 pancreatic adenocarcinomas—for 5-hmC staining. Preoperative biopsies from 16 cases of biliary carcinoma were also stained. Sixteen nonneoplastic/reactive bile duct biopsies served as controls. Results Loss of 5-hmC was seen in 41 of 46 (89.1%) biliary malignancies vs 0 of 20 benign tumors (P  < .001), for a sensitivity and specificity of 89.1% and 100%, respectively. Intrahepatic cholangiocarcinoma showed loss of 5-hmC in 11 of 13 (84.6%) cases, similar to the 30 of 33 (90.9%) cases in other biliary adenocarcinomas (P  = .61). Similarly, 5-hmC loss was more frequent in distal bile duct adenocarcinomas than in pancreatic ductal adenocarcinomas, at 15 of 17 (88.2%) vs 4 of 12 (33.3%), respectively (P  = .0045). There was no difference in the frequency of 5-hmC loss in patients that received neoadjuvant therapy vs those who did not (90.9% vs 88.6%, P   >.99). 5-hmC immunohistochemistry in preoperative biopsies was concordant with the resection specimen in 81.3% (13/16) of cases. Conclusions Loss of 5-hmC is not unique to intrahepatic cholangiocarcinoma among biliary carcinomas, but is a useful diagnostic marker differentiating malignancies of the biliary tree from benign mimics. [ABSTRACT FROM AUTHOR]

Cholangiopathies include a group of chronic progressive disorders, affecting the cholangiocytes, the epithelial cells that line the biliary tree, leading to liver parenchymal fibrosis and eventually end-stage liver disease necessitating transplantation. Experimental modeling of these multifactorial cholestatic diseases faces challenges due to the lack of adequate experimental in vitro and in vivo models. A novel approach employs three-dimensional organoid systems that offer several advantages for modeling disease and testing drug response in vitro. Organoids mimic intercellular communication, replicate the architecture of organs, and maintain the cell's original phenotype. Cholangiocyte organoids provide an in vitro model to study the pathogenesis and pharmacotherapeutic treatment of cholangiopathies and show great promise for regenerative therapies. In particular, patient-derived organoids allow personalized medicine approaches and the study of individual disease characteristics. This review highlights the significance of cholangiocyte organoid models in advancing our understanding of cholangiopathies and driving advancements in regenerative medicine strategies. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Hydatid cysts, primarily found in the liver (70%), are caused by parasitic infections and can lead to severe complications such as cyst rupture. This case report describes a unique instance of a hydatid liver cyst occupying the right lobe with a communicating part with the biliary tree that ruptured showing a concurrent superinfection. Hydatid cysts are a clinical pathology resulting from parasitic infections. They may occur in different organs of the body. However, these are mostly found in the liver (70%). This can cause significant complications including cyst rupture. Several case reports have described various hydatid cyst ruptures; however, only a few have reported an intra‐biliary hydatid cyst rupture. A 24‐year‐old male patient presented with right upper quadrant pain, jaundice, dark urine, and pale stool. Imaging studies, including Magnetic resonance cholangiopancreatography (MRCP) and computed tomography (CT), revealed a beavertail liver, cystobiliary communication and intrahepatic biliary tree‐ruptured hydatid cysts. The cyst was in the right liver lobe, which is the most common site for hydatid cysts. Surgical intervention involving laparoscopic de‐roofing and cyst removal resulted in a smooth recovery without complications. Several case reports have described various hydatid cyst ruptures; however, only a few have reported originally placed intra‐biliary hydatid cyst ruptures. This case report describes a unique instance of a hydatid liver cyst occupying the right lobe with a communicating part with the biliary tree that ruptured showing a concurrent superinfection. [ABSTRACT FROM AUTHOR]

Anatomy has remained an interest of physicians throughout the ages. The biliary tract spans from the liver to the hepatoduodenal mesentery, pancreas, and into the duodenum. Therefore, it is important for not only hepatobiliary surgeons but also general gastrointestinal surgeons, gastroenterologists, radiologists, and pathologists to be familiar with biliary anatomy and its variants. While surgery for hilar cholangiocarcinoma is one of the most challenging procedures, cholecystectomy is one of the most common procedures done from the beginning of surgical training. We hope that by answering the following questions, you will gain a comprehensive understanding of biliary anatomy and a greater appreciation for it. [ABSTRACT FROM AUTHOR]

The gallbladder is not part of the standard survey of a fetus during obstetrical ultrasound. Yet, some anomalies can be detected. Most are clinically benign, but some are features of or associated with more serious complications. Biliary atresia constitutes the diagnosis with the worst prognosis. Its in utero diagnosis is difficult. Still, markers do exist and should be searched for. Knowledge of the normal appearances, variants and abnormalities of the gallbladder contributes to proper management. Any suspicion should lead to meticulous postnatal workup. Pre- or postnatal magnetic resonance imaging can provide additional information in select cases. [ABSTRACT FROM AUTHOR]

Background: Biliary tract cancers (BTCs), encompassing cholangiocarcinoma (CCA), gallbladder (GBC), and ampulla of Vater cancers (AVC), are common hepatobiliary cancer after hepatocellular carcinoma with a high mortality rate. As there is no effective chemopreventive agent to prevent BTCs, this study aimed to explore the role of statins on the risk of BTCs. Methods: PubMed, Embase, and Cochrane Library from inception until 24 April 2020 were searched according to the Meta‐Analyses of Observational Studies in Epidemiology (MOOSE) guidelines. The adjusted risk ratios (aRRs) of BTCs and individual cancer were pooled using a random‐effects model. Results: Eight observational studies (3 cohort and 5 case–control studies) were included with 10,485,231 patients. The median age was 68.0 years (IQR: 67.0–71.5) and 48.3% were male. Statins were associated with a lower risk of all BTCs (aRR: 0.67; 95% CI: 0.51–0.87). The pooled aRR for CCA was 0.60 (95% CI: 0.38–0.94) and GBC was 0.78 (95% CI: 0.68–0.90). There was only one study on AVC with aRR of 0.96 (95% CI: 0.66–1.41). The pooled aRR for lipophilic and hydrophilic statins was 0.78 (95% CI: 0.69–0.88) and 0.70 (95% CI: 0.61–0.80), respectively. The effects were attenuated in studies that adjusted for aspirin and/or non‐steroidal anti‐inflammatory drugs (aRR: 0.80, 95% CI: 0.72–0.89) and metformin (aRR: 0.80, 95% CI: 0.72–0.90). Conclusions: Statins, both lipophilic and hydrophobic, were associated with a lower risk of BTCs, particularly CCA and GBC. [ABSTRACT FROM AUTHOR]

The biliary system is a highly branched tubular network consisting of intrahepatic bile ducts (IHBDs) and extrahepatic bile ducts (EHBDs). IHBDs are derived from hepatic progenitor cells, while EHBDs originate directly from the endoderm through a separate branching morphogenetic process. Traits that are important for cancer are often found to overlap in developmental and other processes. Therefore, it has been suggested that intrahepatic cholangiocarcinomas (iCCAs) and extrahepatic cholangiocarcinomas (eCCAs) have different developmental mechanisms. While much evidence is being gathered on the mechanism of iCCAs, the evidence for eCCA is still very limited. The main reason for this is that there are very few appropriate animal models for eCCA. We can gain important insights from these animal models, particularly genetically engineered mouse models (GEMMs). GEMMs are immunocompetent and mimic human CCA subtypes with a specific mutational pattern, allowing the development of precancerous lesions, that is, biliary intraepithelial neoplasia (BilIN) and intraductal papillary neoplasm of the bile duct (IPNB). This review provides a summary of the pathogenesis and mechanisms of eCCA that can be revealed by GEMMs. Furthermore, we discuss several clinical questions, such as whether BilIN and IPNB really become malignant, whether the peribiliary gland is the origin of eCCAs, and others. [ABSTRACT FROM AUTHOR]

Background: Jaundice within the first 1-2 weeks of a neonate's life will generally self-resolve; however, if it lasts longer than this time frame it warrants further work up. Direct or conjugated hyperbilirubinemia can suggest neonatal cholestasis, which in turn reflects marked reduction in bile secretion and flow. The differential diagnosis for neonatal cholestasis is broad. Neonatal choledocholithiasis is a rare cause of neonatal cholestasis, but should be considered on the differential diagnosis for patients presenting with elevated conjugated bilirubin.Case Presentation: We describe an infant who presented with neonatal cholestasis. He subsequently underwent work up for biliary atresia, as this is one of the more time-sensitive diagnoses that must be made in neonates with conjugated hyperbilirubinemia. He was ultimately found to have choledocholithiasis on magnetic resonance cholangiopancreatography. He was managed conservatively with optimizing nutrition and ursodeoxycholic acid therapy.Conclusions: We found that conservative management, specifically optimizing nutrition and treating with ursodeoxycholic acid, can be a sufficient approach to facilitating resolution of the choledocholithiasis and conjugated hyperbilirubinemia. [ABSTRACT FROM AUTHOR]

Purpose: To compare quantitative biliary measurements obtained with three different magnetic resonance cholangiopancreatography (MRCP) acquisition methods. Methods: This retrospective study was IRB-approved. Patients with combinations of clinically indicated 3D FSE MRCP with sensitivity encoding (SENSE), 3D FSE SENSE MRCP with compressed sensing (CS-FSE; acceleration factor 8), and 3D gradient and spin-echo (GRASE) MRCP, acquired between October 2018 and March 2020, were included. The MRCP + Tuning Threshold algorithm (Perspectum Ltd., Oxford, UK) was used to segment 3D biliary models from MRCP data, with multiple metrics quantified from the models. Single measure, two-way, mixed-effects intra-class correlations, Bland–Altman analyses, and Wilcoxon signed-rank tests were used to compare quantitative measurements. Results: From 160 MRCP datasets (25 3D FSE, 67 3D CS-FSE, 68 3D GRASE) in 69 patients, 48 datasets (7 [28%] 3D FSE, 14 [21%] 3D CS-FSE, 27 [40%] 3D GRASE) failed post-processing due to motion artifacts. The remaining 112 MRCP datasets (18 3D FSE, 53 3D CS-FSE, 41 3D GRASE) from 60 patients were included in the analysis. There was good to excellent agreement between 3D FSE and 3D CS-FSE MRCP for diameter of the left and right hepatic ducts, biliary volume, number and length of ducts, and total length of dilations (ICC: 0.83–0.93). The only metrics that exhibited good agreement between 3D FSE and 3D GRASE MRCP were biliary volume (ICC: 0.75) and total number of dilations (ICC: 0.77). Conclusion: 3D CS-FSE MRCP produces comparable biliary diameter metrics and global duct quantification to 3D FSE MRCP at a significantly reduced acquisition time. [ABSTRACT FROM AUTHOR]

Background Many factors have been incriminated in occurrence of bile duct injuries during laparoscopic cholecystectomy (LC). Misidentification of the anatomy and lack of surgeon's experience seems to be preliminary; however, many techniques were used to avoid such injuries. Aim of the work To asses feasibility of delineation of biliary tree by intracholecystic injection of methylene blue (MB) during LC. Patients and methods A prospective study had been conducted in Ain Shams University Hospitals between January 2019 and January 2020. The study included 60 patients who presented to outpatient department (OPD) with abdominal ultrasound showing chronic calcular cholecystitis and all underwent intracholecystic injection of MB during LC. Results As regards MB delineation of different parts of gall bladder and biliary tract component, gall bladder delineation occurred in all patients (100%), whereas cystic duct delineated in 80% of patients (48/60) and common bile duct (CBD) delineated in 41.7% of patients (25/60) as general and separate assessment of each element alone with no detected cases of bile duct injury. There was significant increase in CBD delineation in patients with single stone and thin-walled gall bladder. Extravasation of dye from the gall bladder into the abdominal cavity occurred and this led to anatomical misidentification and significant prolongation of operative time (extra time was needed for irrigation and suction). Conclusion The technique by which the MB is injected into the lumen of gall bladder to delineate the cystic duct and CBD is feasible, cheap, and performed without any radiation exposure and without using special equipment. By this technique, no cases of biliary injury were detected; however, critical view of safety remains the gold standard surgical technique to prevent bile duct injury. [ABSTRACT FROM AUTHOR]

The study of the fractal architecture of various organs and structures expanded the possibilities for determining the ranges of their functioning and structural arrangement, which, as a result, was reflected in the development of new approaches to diagnostics and therapeutic impacts. The architecture of the excretory duct systems, similar to the hemo‐ and lymph‐ circulatory beds and the bronchial tree, is considered fractal. At the same time, information about hitherto unknown structures of the biliary tree continues to appear in the literature. We aimed to study the features of the spatial geometry of the biliary tree and assess the significance of both its fractal and Euclidean characteristics for the development of approaches that facilitate comprehensive description of intrahepatic biliary tract architecture. We investigated the architecture of the biliary trees of six men, seven male canines, and seven male Wistar rats using the corrosion casting method. Corrosion casts were prepared by injecting solidifying latexes into the bile ducts. The preparations were studied using a light stereomicroscope and a scanning electron microscope. Biliary tree branching is of various types. In addition, the correlation between variations in the caliber and length of the bile ducts and their order is not significant. Therefore, the biliary tree should not be considered as a classical fractal and it consists of the main modules, represented by the network of the bile canaliculi (first nonfractal module) and a biliary tree with a fractal branching (second module) that drains the bile canaliculi mesh and the additional modules represented by the mucosal biliary glands (in mammals with the gallbladder) or the periportal biliary plexus (in mammals without a gallbladder) and the aberrant biliary ducts. Such a configuration of the biliary bed should optimally ensure the smooth implementation of the physiological function of the liver, as well as its adaptation to different pathologies accompanied by biliary hypertension. It also might be considered in the diagnosis and assessment of ductular reaction, biliary regeneration, and/or carcinogenesis. [ABSTRACT FROM AUTHOR]

Background: Infestation with Ascaris lumbricoides is worldwide. About one fourth of the world's population is known to be infected by ascariasis. It is endemic in various parts of Bangladesh. Objectives: To study the various types of clinical presentations, socio-demographic background and sonographic patterns of biliary ascariasis. Results: Mean age (mean ± SD) of presentation was 35.3 ± 15.9 years and female were 80%. Ninety percent of patients were from rural areas and 80% were bearing a lower class socioeconomic background. The most common presentation was upper abdominal pain in 100% of patients. History of worm emesis was present in 34% and previous biliary tree pathology in 24% of patients. Ultrasonography demonstrated the commonest site of worm entrapment was common bile duct in 76% of patients and the commonest pattern was stripe sign pattern in 56% of patients. Conclusion: In endemic countries, ascariasis should to be suspected in patients with biliary disease, especially if there is history of emesis of worm or previous history of biliary tree pathology. Ultrasonography is a reliable, easily available and relatively inexpensive modality of investigation for suspected biliary ascariasis. [ABSTRACT FROM AUTHOR]

Background/Aim: Biliary tree and pancreatic duct can appear in different variations whose proper understanding is obligatory for surgeons. Magnetic resonance cholangiopancreatography (MRCP) is considered a safe and accurate tool for evaluating biliary tree and pancreatic duct. Typical anatomy for right hepatic duct (RHD) and left hepatic duct (LHD) is reported as 57% and 63%, respectively. The most common (4-10%) pancreatic anomaly is divisum. In the present study, we evaluated and determined the prevalence of biliary tree and pancreatic duct variations among patients at a university hospital. Materials and Methods: The MRCP records of 370 patients from 2015 to 2017 were obtained for cross-sectional study. Images were retrospectively reviewed for variations by two independent senior radiologists. Demographic data were obtained for all the patients. Huang et al. classification was used for RHD and LHD variations. The cystic duct was reported based on its course and insertion pattern. The pancreatic duct was observed for the presence of divisum, its course, and configuration. Results: Three hundred and twenty-five patients were included in the final study. Most commonly observed variant for RHD were A1 (34.2%) and A2 (32.2%). For LHD, B1 (71.4%) was the most common variant. Cystic duct insertion was commonly seen as right lateral insertion (27.7%). Pancreatic divisum was observed in 0.6% of cases. Nationality, origin, and gender-specific variations were obtained. Conclusion: Variations in biliary anatomy and pancreatic duct are very diverse and extend from the intrahepatic biliary system down to the pancreas. Performing a similar study on a larger population is mandatory to illustrate the range of variations present within the community. [ABSTRACT FROM AUTHOR]

Injuries to the biliary tree during surgical, endoscopic or invasive radiological diagnostic or therapeutic procedures involving the pancreas, liver or organs of the upper gastrointestinal tract give rise to the need to develop a method for clear discrimination of biliary anatomy from surrounding tissue. Hyperspectral imaging (HSI) is an emerging optical technique in disease diagnosis and image-guided surgery with inherent advantages of being a non-contact, non-invasive, and non-ionizing technique. HSI can produce quantitative diagnostic information about tissue pathology, morphology, and chemical composition. HSI was applied in human liver transplantation and compared to porcine model operations to assess the capability of discriminating biliary anatomy from surrounding biological tissue. Absorbance spectra measured from bile ducts, gall bladder, and liver show a dependence on tissue composition and bile concentration, with agreement between human and porcine datasets. The bile pigment biliverdin and structural proteins collagen and elastin were identified as contributors to the bile duct and gall bladder absorbance spectra. [ABSTRACT FROM AUTHOR]

Contrast enhanced MRI of the liver provides valuable information in the evaluation of both chronic liver disease and focal liver lesions. Currently, two classes of MRI contrast agents are available for clinical use, namely the extracellular contrast agent (ECA) and the hepatobiliary agent (HBA). The use of appropriate contrast agents for liver MRI requires knowledge of the clinical situation and question to be answered. ECAs have been used for decades since their introduction into clinical practice and provide excellent dynamic phase information that is useful in characterizing focal liver lesions. In the last decade, HBAs, particularly Gadoxetate, have been found useful for characterizing lesions with functioning hepatocytes and more importantly in evaluating the biliary tree. Gadoxetate, however, provides less satisfactory dynamic phase images compared to ECAs, particularly during the arterial phase. In this perspective article, we will discuss the various intravenous contrast agents used for liver MRI and their ideal utilization. [ABSTRACT FROM AUTHOR]

Purpose: Autoimmune liver diseases (AILD), including primary sclerosing cholangitis (PSC), autoimmune sclerosing cholangitis (ASC), and autoimmune hepatitis (AIH), have overlapping clinical features but distinct management strategies and outcomes. The purpose of this study was to assess the diagnostic performance of quantitative magnetic resonance cholangiopancreatography (MRCP) parameters for distinguishing PSC/ASC from AIH in children and young adults. Materials and methods: This IRB-approved, cross-sectional study included participants from an institutional AILD registry that underwent baseline serum liver biochemistry testing and 3D fast spin-echo MRCP. The biliary tree was extracted and modeled from MRCP images using novel proprietary software (MRCP+ ™; Perspectum Diagnostics; Oxford, United Kingdom), and quantitative parameters were generated (e.g., biliary tree volume; number and length of bile ducts, strictures, and dilations; bile duct median/maximum diameters). Mann–Whitney U tests were performed to compare laboratory values and MRCP metrics between patient cohorts (clinical diagnosis of PSC/ASC versus AIH). Receiver operating characteristic (ROC) curves and multivariable logistic regression were used to assess diagnostic performance of serum biochemistry values and MRCP parameters for discriminating PSC/ASC from AIH. Results: Thirty percent (14/47) of MRCP exams failed post-processing due to motion artifact. The remaining 33 patients included 20 males and 13 females, with a mean age of 15.1 ± 3.9 years. Eighteen patients were assigned the clinical diagnosis of PSC or ASC and 15 of AIH. All but one quantitative MRCP parameter were significantly different between cohorts (p < 0.05) and predictive of diagnosis (ROC p < 0.05), including numbers of bile duct strictures (area under curve [AUC] = 0.86, p < 0.0001) and dilations (AUC = 0.87, p < 0.0001) and total length of dilated ducts (AUC = 0.89, p < 0.0001). Laboratory values were not significantly different between cohorts (p > 0.05). The best multivariable model for distinguishing PSC/ASC from AIH included total length of dilated ducts (odds ratio [OR], 1.08; 95% CI 1.02–1.14) and maximum left hepatic duct diameter (OR, 1.21; 95% CI 0.57–2.56) [AUC = 0.92]. Conclusion: Quantitative MRCP parameters provide good discrimination of PSC/ASC from AIH. [ABSTRACT FROM AUTHOR]

Duodeno-pancreatic and extrahepatic biliary tree injuries are rare in both adult and pediatric trauma patients, and due to their anatomical location, associated injuries are very common. Mortality is primarily related to associated injuries, but morbidity remains high even in isolated injuries. Optimal management of duodeno-bilio-pancreatic injuries is dictated primarily by hemodynamic stability, clinical presentation, and grade of injury. Endoscopic and percutaneous interventions have increased the ability to non-operatively manage these injuries. Late diagnosis and treatment are both associated to increased morbidity and mortality. Sequelae of late presentations of pancreatic injury and complications of severe pancreatic trauma are also increasingly addressed endoscopically and with interventional radiology procedures. However, for moderate and severe extrahepatic biliary and severe duodeno-pancreatic injuries, immediate operative intervention is preferred as associated injuries are frequent and commonly present with hemodynamic instability or peritonitis. The aim of this paper is to present the World Society of Emergency Surgery (WSES) and American Association for the Surgery of Trauma (AAST) duodenal, pancreatic, and extrahepatic biliary tree trauma management guidelines. [ABSTRACT FROM AUTHOR]

Choledochal cyst is a congenital disease of the biliary tract with extrahepatic and/or intrahepatic biliary tree dilatation. There is a female majority (4:1). Controlling biliary sepsis and curing pancreatitis are prerequisites of surgery. Here, a 35-year-old female patient, came with complaints of epigastric and right upper abdominal pain for three days. On examination per abdomen was soft, non distended, right hypochondriac tenderness was present, no guarding, bowel sounds were audible. Magnetic Resonance Cholangiopancreatography (MRCP) was done which showed fusiform dilatation of Common Bile Duct (CBD), Common Hepatic Duct (CHD), right and left hepatic duct, along with mild dilatation of central intrahepatic biliary radicals (4.8-5 mm)- suggestive of choledochal cyst type IVA. Hepatico-duodenostomy was done. The entire extrahepatic biliary tree should be removed when the choledochal cyst is diagnosed whether or not symptoms are present. Despite being congenital, the condition is typically discovered in adulthood as well. The treatment of choledochal cysts has evolved. The current standard of care is cyst excision and hepaticojejunostomy. [ABSTRACT FROM AUTHOR]

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Objectives: The palliative treatment of cholangiocarcinoma is based on stent placement with well-known procedure-related complications. Consequently, alternative energy-based techniques were put forward with controversial long-term results. This study aims to evaluate the safety and effectiveness of biliary tree laser ablation (LA) in terms of: (i) absence of perforation, (ii) temperature increase, (iii) induced thermal damage in in vivo models. Materials and methods: The common bile duct and cystic ducts of two pigs were ablated with a diode laser (circumferential irradiation pattern) for 6 and 3 min at 7 W. Laser settings were chosen from previous ex vivo experiments. Local temperature was monitored through a fibre Bragg grating (FBG) sensor embedded into the laser delivery probe. Histopathological analysis of the ablated specimen was performed through in situ endomicroscopy, haematoxylin and eosin (H&E) and nicotinamide adenine dinucleotide (NADH) stains. Results: Temperature reached a plateau of 53 °C with consequent thermal damage on the application area, regardless of laser settings and application sites. No perforation was detected macroscopically or microscopically. At the H&E stain, wall integrity was always preserved. The NADH stain allowed to evaluate damage extension. It turned out that the ablation spreading width depended on application time and duct diameter. In situ endomicroscopy revealed a clear distinction between ablated and non-ablated areas. Conclusions: The temperature distribution obtained through LA proved to induce a safe and effective intraductal coagulative necrosis of biliary ducts. These results represent the basis for further experiments on tumour-bearing models for the treatment of obstructive cholangiocarcinoma. [ABSTRACT FROM AUTHOR]

We described magnetic resonance (MR) features of peribiliary metastasis and of periductal infiltrative cholangiocarcinoma. We assessed 35 patients, with peribiliary lesions, using MR 4-point confidence scale. T1-weighted (T1-W), T2-weighted (T2-W) and diffusion-weighted images (DWI) signal intensity, enhancement pattern during arterial, portal, equilibrium and hepatobiliary phase were assessed. We identified 24 patients with periductal-infiltrating cholangiocellular carcinoma. The lesions in 34 patients appeared as a single tissue, while in a single patient, the lesions appeared as multiple individual lesions. According to the confidence scale, the median value was 4 for T2-W, 4 for DWI, 3.6 for T1-W in phase, 3.6 for T1-W out phase, 3 for MRI arterial phase, 3.2 for MRI portal phase, 3.2 for MRI equilibrium phase and 3.6 for MRI hepatobiliary phase. According to Bismuth classification, all lesions were type IV. In total, 19 (54.3%) lesions were periductal, 15 (42.9%) lesions were intraperiductal, and 1 (2.8%) lesion was periductal intrahepatic. All lesions showed hypointense signal in T1-W and in ADC maps and hyperintense signal in T2-W and DWI. All lesions showed a progressive contrast enhancement. There was no significant difference in signal intensity and contrast enhancement among all metastases and among all metastases with respect to CCCs, for all imaging acquisitions (p value >0.05). MRI is the method of choice for biliary tract tumors thanks to the possibility to obtain morphological and functional evaluations. T2-W and DW sequences have highest diagnostic performance. MRI does not allow a correct differential diagnosis among different histological types of metastasis and between metastases and CCC. [ABSTRACT FROM AUTHOR]

Despite decades of basic research, biliary diseases remain prevalent, highly morbid, and notoriously difficult to treat. We have, however, dramatically increased our understanding of biliary developmental biology, cholangiocyte pathophysiology, and the endogenous mechanisms of biliary regeneration and repair. All of this complex and rapidly evolving knowledge coincides with an explosion of new technological advances in the area of regenerative medicine. New breakthroughs such as induced pluripotent stem cells and organoid culture are increasingly being applied to the biliary system; it is only a matter of time until new regenerative therapeutics for the cholangiopathies are unveiled. In this review, the authors integrate what is known about biliary development, regeneration, and repair, and link these conceptual advances to the technological breakthroughs that are collectively driving the emergence of a new global field in biliary regenerative medicine. [ABSTRACT FROM AUTHOR]

Recurrent pyogenic cholangitis (RPC) is an infective process involving the biliary tree typified by pigmented intraductal calculi with dilatation of the intra- and extrahepatic biliary tree. Previously endemic to South-east Asia, RPC can now be seen in Western countries with the increasing access to international travel and immigration. Affected patients are often plagued by recurrent bouts of cholangitis, and commonly suffer from complications such as abscess formation and biliary strictures. In severe cases, cirrhosis with portal hypertension may develop. The disease is also a known risk factor for cholangiocarcinoma, and can be seen in up to 5% of affected patients. Its exact etiology is unknown, but parasitic infections such as Clonorchis sinensis and Ascaris lumbricoides, ascending bacterial infection with gut flora ( Escherichia coli) and low socioeconomic status have been associated strongly with it. This paper reviews the imaging features of the disease, as well as the roles of interventional radiology and surgery with respect to management of the condition. [ABSTRACT FROM AUTHOR]

Peribiliary glands (PBGs) are niches in the biliary tree and containing heterogeneous endodermal stem/progenitors cells that can differentiate, in vitro and in vivo, toward pancreatic islets. The aim of this study was to evaluate, in experimental and human diabetes, proliferation of cells in PBGs and differentiation of the biliary tree stem/progenitor cells (BTSCs) toward insulin-producing cells. Diabetes was generated in mice by intraperitoneal injection of a single dose of 200 mg/kg ( N = 12) or 120 mg/kg ( N = 12) of streptozotocin. Liver, pancreas, and extrahepatic biliary trees were en bloc dissected and examined. Cells in PBGs proliferated in experimental diabetes, and their proliferation was greatest in the PBGs of the hepatopancreatic ampulla, and inversely correlated with the pancreatic islet area. In rodents, the cell proliferation in PBGs was characterized by the expansion of Sox9-positive stem/progenitor cells that gave rise to insulin-producing cells. Insulin-producing cells were located mostly in PBGs in the portion of the biliary tree closest to the duodenum, and their appearance was associated with upregulation of MafA and Gli1 gene expression. In patients with type 2 diabetes, PBGs at the level of the hepatopancreatic ampulla contained cells showing signs of proliferation and pancreatic fate commitment. In vitro, high glucose concentrations induced the differentiation of human BTSCs cultures toward pancreatic beta cell fates. The cells in PBGs respond to diabetes with proliferation and differentiation towards insulin-producing cells indicating that PBG niches may rescue pancreatic islet impairment in diabetes. These findings offer important implications for the pathophysiology and complications of this disease. S tem C ells 2016;34:1332-1342 [ABSTRACT FROM AUTHOR]

Spontaneous or non-traumatic perforation involving the wall of the extrahepatic or intrahepatic biliary tree is a rare entity leading to difficulty and delay in preoperative diagnosis and resulting in 30% to 50% mortality in spite of adequate surgical therapy and postoperative intensive care. Multiple factors are involved in its pathogenesis in both adults and infants. Patients can present with acute or insidious onset. Diagnostic modalities include ultrasound, abdominal CECT and radionuclide hepatobiliary scan. However, in the presence of clinical features of generalized peritonitis, surgery should not be unduly delayed due to non-availability of these diagnostic tools. A high index of suspicion and an early surgical intervention are the mainstays of therapy. Surgical management remains controversial. The nature and extent of surgery, both by open exploration and laparoscopy, should be tailored according to the patient's general condition, the severity of the peritonitis, and intraoperative and cholangiographic findings. [ABSTRACT FROM AUTHOR]

Cholangiocarcinomas (CCAs) are anatomically classified into intrahepatic, perihilar, and distal types. The gross pathological classification of intrahepatic CCAs divides them into mass-forming, periductal-infiltrating, and intraductal-growth types; and perihilar/distal CCAs into flat- and nodular-infiltrating and papillary types. Unique preinvasive lesions appear to precede individual gross types of CCA. Biliary intraepithelial neoplasia, a flat lesion, precedes periductal-, flat-, and nodular-infiltrating CCAs, whereas intraductal papillary neoplasm of the bile duct (IPNB) precedes the intraductal-growth and papillary type of CCAs. IPNBs are heterogeneous in their histological and pathological profiles along the biliary tree. Hepatobiliary cystadenomas/adenocarcinomas are reclassified as cystic IPNBs and hepatic mucinous cystic neoplasms. Peribiliary glands may participate in the development of CCAs. These latest findings present a new challenge for understanding the pathology of CCAs. [ABSTRACT FROM PUBLISHER]

Background and Aims: Although endoscopic ultrasound (EUS) is used in the management of various gastrointestinal (GI) diseases in adults, data on its role in children is limited. This study evaluated the indications, safety, and impact of EUS in children. Methods: Records of children (<18 years age) who underwent EUS between January 2006 and September 2014 were reviewed retrospectively and analyzed. Results: One hundred and twenty-one children (70 males, 51 females) aged 15.2 ± 2.9 years (mean ± SD) underwent 123 diagnostic (including fine needle aspiration cytology (FNAC) in 7) and 2 therapeutic EUS procedures. Conscious sedation was used in 81 procedures (65 %) and general anesthesia in 44 (35 %). The pancreaticobiliary system was evaluated in 114 (118 procedures), mediastinum in 5, and stomach in 2 patients. EUS diagnosed chronic pancreatitis (21 patients), pancreatic necrosis (1), splenic artery pseudoaneurysm (1), gastric varix (1), pseudocysts (3), insulinomas (2), other pancreatic masses (2), choledocholithiasis (2), choledochal cysts (2), portal biliopathy (1), esophageal leiomyoma (1), gastric neuroendocrine tumor (NET) (1), and GI stromal tumor in stomach (1). EUS-guided FNAC was positive in four of seven patients (two had tuberculosis, one pancreatic solid pseudopapillary tumor, and one gastric NET). Three patients had minor adverse events. EUS had a positive clinical impact in 43 (35.5 %) patients. Conclusions: EUS is feasible and safe in children. It provides valuable information that helps in their clinical management. [ABSTRACT FROM AUTHOR]

Liver transplantation and cholangiocarcinoma induce biliary dysfunction following ischemia reperfusion (IR). The function of the intrahepatic biliary tree is regulated by both autocrine and paracrine factors. The aim of the study was to demonstrate that IR-induced damage of cholangiocytes is associated with altered expression of biliary angiogenic factors. Normal and bile duct ligation rats underwent 24-h sham or hepatic reperfusion after 30 min of transient occlusion of the hepatic artery (HAIR) or portal vein (PVIR) before collecting liver blocks and cholangiocyte RNA or protein. We evaluated liver histology, biliary apoptosis, proliferation and expression of VEGF-A/C, VEGFR-2/3, Ang-1/2, and Tie-1/2 in liver sections and isolated small and large cholangiocytes. Normal rat intrahepatic cholangiocyte cultures (NRICC) were maintained under standard conditions in normoxic or under a hypoxic atmosphere for 4 h and then transferred to normal conditions for selected times. Subsequently, we measured changes in biliary proliferation and apoptosis and the expression of VEGF-A/C and VEGFR-2/3. In vivo, HAIR (but not PVIR) induced damage of large bile ducts and decreased proliferation and secretin-stimulated cAMP levels. HAIR-induced damage of large bile ducts was associated with increased expression of VEGF-A/C, VEGFR-2/3, Ang-1/2, and Tie-1/2. In vitro, under hypoxic conditions, there was increased apoptosis and reduced proliferation of NRICC concomitant with enhanced expression of VEGFA/ C and VEGFR-2/3. The functional damage of large bile ducts by HAIR and hypoxia is associated with increased expression of angiogenic factors in small cholangiocytes, presumably due to a compensatory mechanism in response to biliary damage. [ABSTRACT FROM AUTHOR]

Aims To investigate the immunohistochemical expression of dyskerin, a biomarker involved in ribosome production and telomere maintenance, in human fetal, adult and neoplastic bile ducts, and possible correlations with cholangiocarcinoma aggressiveness. Methods and results Sixty consecutive intrahepatic cholangiocarcinomas were collected and used for tissue microarray construction (total: 176 cores); clinical data and follow-up were also collected. Five fetal and 10 normal adult livers were included as controls. Automated immunohistochemistry for dyskerin, p53, and Ki67, and nucleolar silver staining, were performed. In normal livers, dyskerin expression was negative in smaller bile ducts (mean 44.8 μm) and positive in bile ducts of larger diameter (mean 116.1 μm; P < 0.001). Expression was positive in 56.7% of cholangiocarcinomas, and correlated with p53 mutation ( P = 0.008) and a higher proliferative (Ki67) index ( P = 0.003), which were included as markers of tumour aggressiveness. Finally, dyskerin-positive cholangiocarcinomas showed a negative trend in disease-free survival ( P = 0.078) on univariate analysis. Conclusions The non-neoplastic biliary tree seems to progressively lose dyskerin expression from the major branches to the peripheral portal bile ducts. Similarly, intrahepatic cholangiocarcinomas showed two patterns of dyskerin expression, and the dyskerin-positive phenotype seemed to characterize more aggressive cholangiocarcinomas. [ABSTRACT FROM AUTHOR]

Routine experiences suggest that cholangiocarcinomas (CCAs) show different clinicopathological behaviors along the biliary tree, and hilar CCA apparently resembles pancreatic duct adenocarcinoma (PDAC). Herein, the backgrounds for these similarities were reviewed. While all cases of PDAC, hilar CCA, intrahepatic CCA (ICCA) and CCA components of combined hepatocellular‐cholangiocarcinoma (cHC‐CCA) were adenocarcinomas, micropapillary patterns and columnar carcinoma cells were common in PDAC and hilar CCA, and trabecular components and cuboidal carcinoma cells were common in ICCA and CCA components of cHC‐CCA. Anterior gradient protein‐2 and S100P were frequently expressed in perihilar CCA and PDAC, while neural cell adhesion molecule and luminal epithelial membrane antigen were common in CCA components of c‐HC‐CCA. Pdx1 and Hes1 were frequently and markedly expressed aberrantly in PDAC and perihilar CCA, although their expression was rare and mild in CCA components in cHC‐CCA and ICCA. Hilar CCA showed a similar postoperative prognosis to PDAC but differed from ICCA and cHC‐CCA. Taken together, hilar CCA may differ from ICCA and CCA components of cHC‐CCA but have a similar development to PDAC. These similarities may be explained by the unique anatomical, embryological and reactive nature of the pancreatobiliary tract. Further studies of these intractable malignancies are warranted. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate MDCT findings of 1-2-cm sized gallbladder (GB) polyps for differentiation between benign and malignant polyps. Methods: Institutional review board approval was obtained, and informed consent was waived. Portal venous phase CT scans of 1-2-cm sized GB polyps caused by various pathologic conditions were retrospectively reviewed by two blinded observers. Among the 36 patients identified, 21 had benign polyps with the remaining 15 having malignant polyps. Size, margin, and shape of GB polyps were evaluated. Attenuation values of the polyps, including mean attenuation, maximum attenuation, and standard deviation, were recorded. As determined by visual inspection, the degree of polyp enhancement was evaluated. Using these CT findings, each of the two radiologists assessed and recorded individual diagnostic confidence for differentiating benign versus malignant polyps on a 5-point scale. The diagnostic performance of CT was evaluated using a receiver operating characteristic curve analysis. Results: There was no significant difference in size between benign and malignant GB polyps. Ill-defined margin and sessile morphology were significantly associated with malignant polyp. There was a significant difference in mean and maximum attenuation values between benign and malignant GB polyps. Mean standard deviation value of malignant polyps was significantly higher than that of benign polyps. All malignant polyps showed either hyperenhancement or marked hyperenhancement. A value for the diagnosis of malignant GB polyps was 0.905. Conclusion: Margin, shape, and enhancement degree are helpful in differentiating between benign and malignant polyps of 1-2-cm sizes. [ABSTRACT FROM AUTHOR]

The bile duct of Luschka (BDL) is an anatomic anomaly that is an important cause of bile leakage after bile duct surgery. We report a case of bile duct carcinoma with dilated BDL that was diagnosed by ultrasonography (US). An 83-year-old man presented with an obstructive jaundice. US on admission revealed the presence of a solid hypoechoic mass in the bile duct at the hepatic duct confluence and a branch of the bile duct, about 2-4 mm in diameter, distinct from the dilated right anterior hepatic duct slightly upstream of the tumor. This branch had a spiral structure, extended along the gallbladder bed on the surface of segment 5 (S5) of the liver, and emanated small branches that entered the hepatic parenchyma. There has been no previous report of delineation of BDL by preoperative US. © 2012 Wiley Periodicals, Inc. J Clin Ultrasound 41:558-562, 2013. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the added role of T1-weighted (T1w) gadolinium ethoxybenzyl diethylenetriamine penta-acetic acid (Gd-EOB-DTPA)-enhanced magnetic resonance cholangiography (MRC) compared with T2-weighted MRC (T2w-MRC) in the detection of biliary leaks. Methods: Ninety-nine patients with suspected biliary complications underwent routine T2w-MRC and T1w contrast-enhanced (CE) MRC using Gd-EOB-DTPA to identify biliary leaks. Two observers reviewed the image sets separately and together. MRC findings were compared with those of surgery and percutaneous transhepatic cholangiopancreatography. The sensitivity, specificity and accuracy of the techniques in identifying biliary leaks were calculated. Results: Accuracy of locating biliary leaks was superior with the combination of Gd-EOB-DTPA-enhanced MRC and T2w-MRC ( P < 0.05).The mean sensitivities were 79 % vs 59 %, and the mean accuracy rates were 84 % vs 58 % for combined CE-MRC and T2w-MRC vs sole T2w-MRC. Nineteen out of 21 patients with biliary-cyst communication, 90.4 %, and 12/15 patients with post-traumatic biliary extravasations, 80 %, were detected by the combination of Gd-EOB-DTPA-enhanced MRC and T2w-MRC images, P < 0.05. Conclusions: Gd-EOB-DTPA-enhanced MRC yields information that complements T2w-MRC findings and improves the identification and localisation of the bile extravasations (84 % accuracy, 100 % specificity, P < 0.05). We recommend Gd-EOB-DTPA-enhanced MRC in addition to T2w-MRC to increase the preoperative accuracy of identifying and locating extravasations of bile. Key Points: • Magnetic resonance cholangiography (MRC) does not always detect bile leakage and cysto-biliary communications. • Gd-EOB-DTPA-enhanced MRC helps by demonstrating extravasation of contrast material into fluid collections. • Gd-EOB-DTPA-enhanced MRC also demonstrates the leakage site and bile duct injury type. • Combined Gd-EOB-DTPA-enhanced and T2w-MRC can provide comprehensive information about biliary system. • Gd-EOB-DTPA-enhanced MRC is non-invasive and does not use ionising radiation. [ABSTRACT FROM AUTHOR]

Aim-Background: Choledochal cysts are rare entities presenting as cystic dilatations of the biliary tree. Even though their origin is uncertain, they are usually referred for removal because of malfunction of the biliary tract as well as their association with developing malignancy. Traditionally, these choledochal cysts are classified under 5 main types. A condition that is even rarer, the cystic duct cyst, is not included in this classification. We describe such a case of a cyst of the cystic duct that was successfully removed via laparoscopy. Material-Method: A 32-year-old patient is described who was admitted to the hospital complaining of symptoms consistent with acute cholecystitis and associated with signs of biliary tract obstruction. Following imaging investigation with MRI, a cystic duct malformation was revealed and further surgical treatment was planned. Results-Conclusion: Our case shows that even though cystic duct cysts are extremely rare, they are a clinical entity that cannot be ignored and needs to be recognized. Given the existence of similar case reports in literature and the similarity of cystic duct cysts with other choledochal cysts, a new category in the Todani classification may be considered so that clinicians are prepared for this 'Type VI' cystic variant. [ABSTRACT FROM AUTHOR]

Background/Aims: An end-to-side biliodigestive anastomosis is the most common procedure performed in hepato-pancreato-biliary surgery, and this procedure may become technically demanding. A telescopic ureterovesical anastomosis is frequently used in transplant surgery. The aim of this study was to investigate the feasibility of constructing a telescopic biliodigestive anastomosis. Methodology: The technique-standardization ( n = 8) and main study ( n = 3) groups were formed from 11 pigs. A single-stitch telescopic anastomosis with a self-disposable internal stent was performed in the main study group. The animals were sacrificed at the end of the 4-week follow-up period, and cholangiograms and tissue samples were obtained. Repeated biological, hematological, and biochemical data were recorded. Results: No bilomas or functional biliary strictures were identified in any of the main study group animals. Light microscopy revealed intestinal metaplasia of the biliary epithelium in the portion of the bile duct telescoped inside the intestinal lumen. Conclusions: Telescopic bilioenterostomy with a single pull-through stitch and a self-disposable stent is quick and reproducible in animal models, and it appears to be free of complications. Further experiments with longer follow-up periods are required to confirm that this anastomotic technique does not lead to episodes of delayed cholangitis or development of adenocarcinoma. [ABSTRACT FROM AUTHOR]

The anterior gradient protein-2 (AGR2) is overexpressed in numerous tumours such as breast, prostate or pancreas carcinomas and recently reported in fibrolamellar hepatocellular carcinoma (HCC). In the present study, we further describe AGR2 expression patterns all along the adult and fetal biliary tree and in cholangiocarcinomas. Immunohistochemistry using anti-AGR2 antibodies was performed in adult and fetal livers, gallbladders as well as cholangiocarcinomas and HCCs. In adult and fetal liver, the tall epithelial cells covering the large bile ducts as well as gallbladder epithelial cells showed strong AGR2 staining. Hilar and extrahepatic cholangiocarcinomas, and a subset of intrahepatic cholangiocarcinomas, which displayed a morphological mucus-excreting feature, also displayed AGR2 expression. In contrast, AGR2 expression was not detected in typical HCCs. Our study shows that the differential expression of AGR2 is a phenotypic feature of the cholangiocytes covering different segments of the biliary tree. This pattern of expression is conserved during fetal and adult life, as well as during biliary carcinogenesis. [ABSTRACT FROM AUTHOR]

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Intraductal papillary neoplasm of the bile duct (IPNB) is characterized by exophytic proliferation of neoplastic epithelial cells with fibrovascular stalks in bile duct lumen, mucin hypersecretion, and considerable dilatation or multilocular changes of the affected bile ducts. A mucin-producing bile duct tumor is an IPNB with excessive mucin production and clinical symptoms. Herein, the phenotypes as well as the tumorigenesis and progression of IPNB are reviewed with immunohistochemical assistance. The tumors are subdivided into three phenotypes: pancreatobiliary, intestinal, and gastric. About half of IPNB cases are of the pancreatobiliary type, and the remaining half are of the intestinal type. Aberrant expression of CDX2 with MUC2 and CK20 is related to the development of intestinal metaplasia. Inactivation of P16INK4a and nuclear expression of β-catenin are related to the development of IPNB. Decreased expression of membranous β-catenin and E-cadherin and aberrant expression of MMP-7 and -9 and of MUC1 are related to invasion of IPNB with tubular adenocarcinoma, whereas MUC2 is involved in the invasion of IPNB with mucinous carcinoma. IPNB can be regarded as a counterpart of intraductal papillary mucinous neoplasm (IPMN) of the pancreas, particularly the main duct type. More comparative studies between IPNB and pancreatic IPMN are recommended for further analysis of these papillary neoplasms. [ABSTRACT FROM AUTHOR]

The development of multidetector row computed tomography (MDCT) has led to the acquisition of true isotropic voxels that can be postprocessed to yield images in any plane of the same resolution as the original axially acquired images. This, coupled with rapid MDCT imaging during peak target organ enhancement has led to a variety of means to review imaging information beyond that of the axial perspective. Postprocessing can be utilized to identify variant biliary anatomy to guide preoperative planning of biliary-related surgery, determine the level and cause of biliary obstruction and assist in staging of biliary cancer. Postprocessing can also be used to identify pancreatic ductal variants, visualize diagnostic features of pancreatic cystic lesions, diagnose and stage pancreatic cancer, and differentiate pancreatic from peripancreatic disease. [ABSTRACT FROM AUTHOR]

Sclerosing cholangitis is heterogeneous in its etiopathogenesis. Recently, sclerosing cholangitis showing abundant immunoglobulin (Ig)G4+ plasma cell infiltration was added to the sclerosing cholangitis group. This form was frequently associated with sclerosing pancreatitis (autoimmune pancreatitis) and also occasionally with other diseases such as chronic sclerosing sialadenitis, all of which falls within IgG4-related sclerosing disease. Herein, this new member, called IgG4-related sclerosing cholangitis (IgG4-SC), is reviewed. IgG4-SC shows grossly medullary and fleshy lesions along the biliary tree, and histologically marked lymphoplasmacytic infiltration with extensive fibrosis, and obliterative phlebitis, sharing histopathological features with sclerosing pancreatitis. Peribiliary glands are also severely affected. Interestingly, hepatic inflammatory pseudotumor (HIP) is not infrequently associated with IgG4-SC, and is thought as a local exaggeration of IgG4-SC. Immunohistochemically, many IgG4+ plasma cells and CD4+/CD25+ regulatory T cells are found around the affected bile ducts and portal tracts. Incontrast, these cells are scarce in the affected bile ducts of primary sclerosing cholangitis (PSC), a prototype of sclerosing cholangitis. Biliary lining epithelia are relatively spared in IgG4-SC in comparison with those of PSC showing degeneration and ulceration. In some cases of IgG4-SC, IgG4+ plasma cells are also found considerably in small portal tracts, so needle liver biopsy is useful for the diagnosis of IgG4-SC. Therapeutically, IgG4-SC responds well to steroid therapy, while such character is not reported in PSC. Taken together, IgG4-SC may be etiologically different from PSC, and immunopathological processes relating to IgG4 and regulatory T cells may be involved in the pathogenesis of IgG4-SC. Further studies are needed to clarify the etiopathogenesis of IgG4-SC and its related disorders. [ABSTRACT FROM AUTHOR]

Aims: The histogenesis and biological behaviour of peripheral intrahepatic cholangiocarcinoma (peripheral CC) remain unclarified. The aim of this study was to examine the growth pattern of peripheral CC (24 cases) in comparison with hepatocellular carcinoma (HCC, 27 cases) and metastatic colorectal adenocarcinoma (MCA, 24 cases). Methods and results: Tumour/surrounding liver borders were classified as: (i) fibrous encapsulation, (ii) compressive growth, and (iii) infiltrating replacement. Nineteen of 24 peripheral CCs showed (iii), whereas 23 of 27 HCCs showed (i) and 17 of 24 MCAs showed (ii). In (iii), carcinoma cells infiltrated the surrounding liver without compression, and hepatic supporting vascular structures such as portal tracts were secondarily incorporated into the tumour. In (i) and (ii), the surrounding liver was compressed and no or few portal tracts were incorporated within the tumour. Fifteen of 24 peripheral CCs were composed of carcinoma cells resembling reactive bile ductules and these cells were positive for neural cell adhesion molecule (NCAM), a marker of proliferating bile ductules. The remaining nine peripheral CCs were composed of ordinary adenocarcinoma and negative for NCAM. Conclusions: A subgroup of peripheral CCs with an infiltrating replacement growth pattern resembles reactive bile ductules and expresses NCAM. ‘Bile ductular carcinoma’ may be a better term for this subgroup. [ABSTRACT FROM AUTHOR]

Objective: Common bile duct (CBD) brushings have been recognized as a technique of moderate sensitivity and high specificity in identifying carcinoma of the ampulla and pancreatico-biliary regions. This study evaluated the increase in sensitivity of this technique using the ThinPrep® technique of specimen preparation when compared with conventional cytology smears. Methods: A total of 113 bile duct brushings were included in the study (38 conventional smears and 75 slides prepared using the ThinPrep® technique). All slides were reviewed by one cytologist. Five categories of reporting were used: inadequate, negative, atypia, suspicious and malignant. Results: The inadequate category of reporting disappeared in the ThinPrep® group with improved specimen fixation and preparation and hence reduced artefact. Sensitivity of diagnosis of malignancy increased from 39% in conventional smears to 53% in the ThinPrep® group. Specificity, positive and negative predictive values and accuracy were 100%, 100%, 60% and 68% for conventional smears and were 100%, 100%, 60% and 72%, respectively, for ThinPrep® specimens. Conclusions: ThinPrep® technique was associated with increased sensitivity of diagnosis, in part due to improved specimen fixation and reduced artefact. Cytology of bile duct brushings is an important diagnostic tool for sites from which it can be difficult to obtain a histology biopsy. It may therefore provide the only opportunity for tissue diagnosis of carcinoma from these sites, hence the importance of optimizing sensitivity. [ABSTRACT FROM AUTHOR]

Expression of the novel protein tyrosine phosphatase interacting protein 51 (PTPIP51) was investigated on mRNA and protein level in the liver of adult Wistar rats. The presence of PTPIP51 mRNA was detected by Northern blotting. Immunostaining showed expression of PTPIP51 protein in distinct non-parenchymal cells. These cells were identified as Kupffer cells, stellate cells and natural killer cells by detection of cell-specific antigens. Whereas most endothelial cells lining large vessels reacted positive to the PTPIP51 antibody, sinusoidal endothelium showed no detectable amount of PTPIP51. Furthermore, PTPIP51 was also found to be expressed in cells forming the biliary tree. An additional subcellular analysis of the non-parenchymal cells by means of electron microscopy showed the presence of PTPIP51 protein in the cytoplasm and in the nuclei of non-parenchymal cells. Most of the hepatocytes did not show any immuno-detectable amount of PTPIP51, yet, some revealed PTPIP51 protein either in the cytoplasm or in the nucleus. [ABSTRACT FROM AUTHOR]

In cases where the papilla of Vater is unreachable because of pyloric/duodenal stenosis, or a catheter cannot be introduced into the papilla, or with recurrent tumor growth, or after previous gastrointestinal surgery, percutaneous transhepatic cholangiodrainage (PTCD) is considered to be the therapeutic alternative in cholestasis. The purpose of this report was to demonstrate that endoscopic ultrasound (EUS)-guided transesophageal cholangiodrainage is a feasible alternative in patients who decline to undergo PTCD. A 67-year-old female patient with recurrent tumor growth at the hepaticojejunostomy 17 months after a formerly resected cholangiocarcinoma (pT3, pN0 (0/2), M0, G2, R0; extended right hemihepatectomy), cholangitis, and peritoneal carcinomatosis underwent an EUS-guided transesophageal procedure to obtain cholangiodrainage by (i) puncture of a branch of the biliary tree at the left hepatic site, (ii) insertion of a guide wire into the bile duct and the anastomosed jejunum using the rendezvous technique with endoscopic retrograde cholangiopancreatography (ERCP)/conventional endoscopy, (iii) transesophagohepatic placement of an 8.5-Fr. double pigtail catheter, and (iv) transhepatic placement of a Wallstent through the jejunal stenosis, resulting in complete alleviation of the biliary and jejunal obstruction. There were no severe complications such as perforation or bleeding and no stent occlusion within the patient's lifetime of more than 3 months. Death was related to progressive tumor growth. EUS-guided transesophageal cholangiodrainage, here described in combination with Wallstent placement, is a reasonable, feasible, and encouraging treatment alternative in selected patients where conventional ERCP or PTCD is not an option. [ABSTRACT FROM AUTHOR]

Rhabdomyosarcoma (RMS) is the most common tumour of the biliary tree in childhood. In children, it is a rare lesion, accounting for about 1% of all RMS. Hepatobiliary botryoid RMS is a disease affecting young children at a median age of about 3 years. In literature, the radiological findings of hepatobiliary RMS have been described in small series and some case reports. In this case report, we present a rare case of RMS of the extrahepatic biliary tree initially diagnosed as a choledochal cyst. [ABSTRACT FROM AUTHOR]

Objective: Endoscopic retrograde cholangiopancreaticography (ERCP)-guided brushing has been the standard of practice for surveillance and detection of carcinoma in the biliary tree. Few studies have evaluated the role of endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) in diagnosing clinically suspected cholangiocarcinoma. The role of this method in diagnosing clinically suspected gallbladder malignancies has not been extensively evaluated in the USA. This study investigates the role of EUS-FNA in the diagnosis of clinically suspected biliary tree and gallbladder malignancies in a large patient series. Methods: EUS-FNAs were obtained from 46 bile duct and seven gallbladder lesions. On-site rapid interpretation was provided using air-dried Diff Quik stained smears. In addition, alcohol fixed Papanicoloau stained smears and Thin Prep preparations ( Cytye Corp., Marlborough, MA, USA) were evaluated before providing a final cytological diagnosis. Tissue biopsies and/or clinical follow-up were used as the standards to determine operating characteristics for EUS-FNA. Results: The mean ages for bile duct and gallbladder lesions were 66 years (range: 37–84 years), and 69 years (range 49–86 years), respectively. All cases diagnosed as suspicious/malignant on preliminary evaluation were confirmed on final cytological interpretation (27/27). The operating characteristics show that EUS-FNA is highly specific (100%) with sensitivity rates of 87% and 80% from clinically suspected malignancies of biliary tract and gallbladder, respectively. Sampling error in three cases and associated acute inflammation in two cases resulted in false-negative diagnoses. Conclusions: EUS-FNA of biliary tree and gallbladder carcinoma is highly specific and should be considered for evaluation of clinically suspicious lesions. Marked inflammation may result in false-negative diagnoses. [ABSTRACT FROM AUTHOR]

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Hydatid disease oldie liver is a parasitic infection. Surgery still remains as the primary choice of treatment. Caustic sclerosing cholangitis is reported following surgical treatment. Hypertonic saline (20%), povidone iodine (1%), and silver nitrate (0.5%) are extensively used as scolicidal solutions. The effects of these scolicidal agents on liver and biliary tree are investigated by direct injection into the common bile duct of rats. At the end of 15 wk, liver function tests, cholangiography, and histopathological examination of the liver and biliary tree were performed. Liver function tests were within normal limits, except elevation of alkaline phosphatase in 2 and 1 rats of the silver nitrate and povidone iodine groups, respectively. Differences were not statistically significant (p > .05). Cholangiograms of the rats in all groups were normal. Histopathologic changes comprising low-grade inflammatory changes were induced in all study groups. The intensity of the lesions were more remarkable with silver nitrate and minimal with hypertonic saline. We suggest that direct injection of scolicidal agents into the cyst should be avoided particularly in case of biliary communication. If this is to be practiced, hypertonic saline should be preferred as a scolicidal agent. [ABSTRACT FROM AUTHOR]

The arrival of new surgical transplantation techniques, such as split living donor or auxiliary liver transplantation, have increased the incidence of vascular and biliary complications. The causes, symptoms, and diagnostic modalities of arterial, portal caval, and biliary complications are detailed. Interventional techniques, such as balloon angioplasty and stent placement in the arterial and portal tree, as well as biliary interventional techniques, are discussed. [ABSTRACT FROM AUTHOR]