Biliary Tract Cancer*
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Cholangiocarcinoma 2020: the Next Horizon in Mechanisms and Management
CONSENSUS STATEMENT Cholangiocarcinoma 2020: the next horizon in mechanisms and management Jesus M. Banales 1,2,3 ✉ , Jose J. G. Marin 2,4, Angela Lamarca 5,6, Pedro M. Rodrigues 1, Shahid A. Khan7, Lewis R. Roberts 8, Vincenzo Cardinale9, Guido Carpino 10, Jesper B. Andersen 11, Chiara Braconi 12, Diego F. Calvisi13, Maria J. Perugorria1,2, Luca Fabris 14,15, Luke Boulter 16, Rocio I. R. Macias 2,4, Eugenio Gaudio17, Domenico Alvaro18, Sergio A. Gradilone19, Mario Strazzabosco 14,15, Marco Marzioni20, Cédric Coulouarn21, Laura Fouassier 22, Chiara Raggi23, Pietro Invernizzi 24, Joachim C. Mertens25, Anja Moncsek25, Sumera Rizvi8, Julie Heimbach26, Bas Groot Koerkamp 27, Jordi Bruix2,28, Alejandro Forner 2,28, John Bridgewater 29, Juan W. Valle 5,6 and Gregory J. Gores 8 Abstract | Cholangiocarcinoma (CCA) includes a cluster of highly heterogeneous biliary malignant tumours that can arise at any point of the biliary tree. Their incidence is increasing globally, currently accounting for ~15% of all primary liver cancers and ~3% of gastrointestinal malignancies. The silent presentation of these tumours combined with their highly aggressive nature and refractoriness to chemotherapy contribute to their alarming mortality, representing ~2% of all cancer-related deaths worldwide yearly. The current diagnosis of CCA by non-invasive approaches is not accurate enough, and histological confirmation is necessary. Furthermore, the high heterogeneity of CCAs at the genomic, epigenetic and molecular levels severely compromises the efficacy of the available therapies. In the past decade, increasing efforts have been made to understand the complexity of these tumours and to develop new diagnostic tools and therapies that might help to improve patient outcomes. -
Primary Hepatic Carcinoid Tumor with Poor Outcome Om Parkash Aga Khan University, [email protected]
eCommons@AKU Section of Gastroenterology Department of Medicine March 2016 Primary Hepatic Carcinoid Tumor with Poor Outcome Om Parkash Aga Khan University, [email protected] Adil Ayub Buria Naeem Sehrish Najam Zubair Ahmed Aga Khan University See next page for additional authors Follow this and additional works at: https://ecommons.aku.edu/ pakistan_fhs_mc_med_gastroenterol Part of the Gastroenterology Commons Recommended Citation Parkash, O., Ayub, A., Naeem, B., Najam, S., Ahmed, Z., Jafri, W., Hamid, S. (2016). Primary Hepatic Carcinoid Tumor with Poor Outcome. Journal of the College of Physicians and Surgeons Pakistan, 26(3), 227-229. Available at: https://ecommons.aku.edu/pakistan_fhs_mc_med_gastroenterol/220 Authors Om Parkash, Adil Ayub, Buria Naeem, Sehrish Najam, Zubair Ahmed, Wasim Jafri, and Saeed Hamid This report is available at eCommons@AKU: https://ecommons.aku.edu/pakistan_fhs_mc_med_gastroenterol/220 CASE REPORT Primary Hepatic Carcinoid Tumor with Poor Outcome Om Parkash1, Adil Ayub2, Buria Naeem2, Sehrish Najam2, Zubair Ahmed, Wasim Jafri1 and Saeed Hamid1 ABSTRACT Primary Hepatic Carcinoid Tumor (PHCT) represents an extremely rare clinical entity with only a few cases reported to date. These tumors are rarely associated with metastasis and surgical resection is usually curative. Herein, we report two cases of PHCT associated with poor outcomes due to late diagnosis. Both cases presented late with non-specific symptoms. One patient presented after a 2-week history of symptoms and the second case had a longstanding two years symptomatic interval during which he remained undiagnosed and not properly worked up. Both these cases were diagnosed with hepatic carcinoid tumor, which originates from neuroendocrine cells. Case 1 opted for palliative care and expired in one month’s time. -
Progress Report Cholestasis and Lesions of the Biliary Tract in Chronic Pancreatitis
Gut: first published as 10.1136/gut.19.9.851 on 1 September 1978. Downloaded from Gut, 1978, 19, 851-857 Progress report Cholestasis and lesions of the biliary tract in chronic pancreatitis The occurrence of jaundice in the course of chronic pancreatitis has been recognised since the 19th century" 2. But in the early papers it is uncertain whether the cases were due to acute, acute relapsing, or to chronic pan- creatitis, or even to pancreatic cancer associated with pancreatitis or benign ampullary stenosis. With the introduction of endoscopic retrograde cholangiopancreato- graphy (ERCP), there has been a renewed interest in the biliary complica- tions of chronic pancreatitis (CP). However, papers published recently by endoscopists have generally neglected the cholangiographic aspect of the lesions and are less precise and less well documented than papers published just after the second world war, following the introduction of manometric cholangiography3-5. Furthermore, the description of obstructive jaundice due to chronic pancreatitis, classical 20 years ago, seems to have been forgotten until the recent papers. Radiological aspects of bile ducts in chronic pancreatitis http://gut.bmj.com/ If one limits the subject to primary diseases of the pancreas, particularly chronic calcifying pancreatitis (CCP)6, excluding chronic pancreatitis secondary to benign ampullary stenosis7, cancer obstructing the main pancreatic duct8 9 and acute relapsing pancreatitis secondary to gallstones'0 radiological aspect of the main bile duct" is type I the most.common on September 25, 2021 by guest. Protected copyright. choledocus (Figure). This description has been repeatedly confirmed'2"13. It is a long stenosis of the intra- or retropancreatic part of the main bile duct. -
Liver, Gallbladder, Bile Ducts, Pancreas
Liver, gallbladder, bile ducts, pancreas Coding issues Otto Visser May 2021 Anatomy Liver, gallbladder and the proximal bile ducts Incidence of liver cancer in Europe in 2018 males females Relative survival of liver cancer (2000 10% 15% 20% 25% 30% 35% 40% 45% 50% 0% 5% Bulgaria Latvia Estonia Czechia Slovakia Malta Denmark Croatia Lithuania N Ireland Slovenia Wales Poland England Norway Scotland Sweden Netherlands Finland Iceland Ireland Austria Portugal EUROPE - Germany 2007) Spain Switzerland France Belgium Italy five year one year Liver: topography • C22.1 = intrahepatic bile ducts • C22.0 = liver, NOS Liver: morphology • Hepatocellular carcinoma=HCC (8170; C22.0) • Intrahepatic cholangiocarcinoma=ICC (8160; C22.1) • Mixed HCC/ICC (8180; TNM: C22.1; ICD-O: C22.0) • Hepatoblastoma (8970; C22.0) • Malignant rhabdoid tumour (8963; (C22.0) • Sarcoma (C22.0) • Angiosarcoma (9120) • Epithelioid haemangioendothelioma (9133) • Embryonal sarcoma (8991)/rhabdomyosarcoma (8900-8920) Morphology*: distribution by sex (NL 2011-17) other other ICC 2% 3% 28% ICC 56% HCC 41% HCC 70% males females * Only pathologically confirmed cases Liver cancer: primary or metastatic? Be aware that other and unspecified morphologies are likely to be metastatic, unless there is evidence of the contrary. For example, primary neuro-endocrine tumours (including small cell carcinoma) of the liver are extremely rare. So, when you have a diagnosis of a carcinoid or small cell carcinoma in the liver, this is probably a metastatic tumour. Anatomy of the bile ducts Gallbladder -
Slug Overexpression Is Associated with Poor Prognosis in Thymoma Patients
306 ONCOLOGY LETTERS 11: 306-310, 2016 Slug overexpression is associated with poor prognosis in thymoma patients TIANQIANG ZHANG, XU CHEN, XIUMEI CHU, YI SHEN, WENJIE JIAO, YUCHENG WEI, TONG QIU, GUANZHONG YAN, XIAOFEI WANG and LINHAO XU Department of Thoracic Surgery, The Affiliated Hospital, Qingdao University, Qingdao, Shandong 266003, P.R. China Received November 4, 2014; Accepted May 22, 2015 DOI: 10.3892/ol.2015.3851 Abstract. Slug, a member of the Snail family of transcriptional previously been regarded as a benign disease, but more recent factors, is a newly identified suppressive transcriptional factor evidence indicated that it is a potentially malignant tumor of E‑cadherin. The present study investigated the expression requiring prolonged follow‑up (4). However, biomarkers for pattern of Slug in thymomas to evaluate its clinical significance. thymoma diagnosis and prognosis have not yet been estab- Immunohistochemistry was used to investigate the expression lished. pattern of the Slug protein in archived tissue sections from Slug is a member of the Snail family of zinc‑finger tran- 100 thymoma and 60 histologically normal thymus tissue scription factors and was first identified in the neural crest and samples. The associations between Slug expression and developing mesoderm of chicken embryos (5). Slug induces the clinicopathological factors, such as prognosis, were analyzed. downregulation of E-cadherin, an adhesion molecule, leading Positive expression of Slug was detected in a greater propor- to the breakdown of cell-cell adhesions and the acquisition of tion of thymoma samples [51/100 (51%) patients, P<0.001] invasive growth properties in cancer cells (6). These changes compared with normal thymus tissues [9/60 (15%) cases]. -
Cholangiocarcinoma Associated With
Schmidt et al. Journal of Medical Case Reports (2016) 10:200 DOI 10.1186/s13256-016-0989-1 CASE REPORT Open Access Cholangiocarcinoma associated with limbic encephalitis and early cerebral abnormalities detected by 2-deoxy-2- [fluorine-18]fluoro-D-glucose integrated with computed tomography-positron emission tomography: a case report Sergio L. Schmidt1,2,3*, Juliana J. Schmidt1,2, Julio C. Tolentino2, Carlos G. Ferreira4,5, Sergio A. de Almeida6, Regina P. Alvarenga2, Eunice N. Simoes2, Guilherme J. Schmidt2, Nathalie H. S. Canedo7 and Leila Chimelli7 Abstract Background: Limbic encephalitis was originally described as a rare clinical neuropathological entity involving seizures and neuropsychological disturbances. In this report, we describe cerebral patterns visualized by positron emission tomography in a patient with limbic encephalitis and cholangiocarcinoma. To our knowledge, there is no other description in the literature of cerebral positron emission tomography findings in the setting of limbic encephalitis and subsequent diagnosis of cholangiocarcinoma. Case presentation: We describe a case of a 77-year-old Caucasian man who exhibited persistent cognitive changes 2 years before his death. A cerebral scan obtained at that time by 2-deoxy-2-[fluorine-18]fluoro-D-glucose integrated with computed tomography-positron emission tomography showed low radiotracer uptake in the frontal and temporal lobes. Cerebrospinal fluid analysis indicated the presence of voltage-gated potassium channel antibodies. Three months before the patient’s death, a lymph node biopsy indicated a cholangiocarcinoma, and a new cerebral scan obtained by 2-deoxy-2-[fluorine-18]fluoro-D-glucose integrated with computed tomography- positron emission tomography showed an increment in the severity of metabolic deficit in the frontal and parietal lobes, as well as hypometabolism involving the temporal lobes. -
Guideline for the Evaluation of Cholestatic Jaundice
CLINICAL GUIDELINES Guideline for the Evaluation of Cholestatic Jaundice in Infants: Joint Recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition ÃRima Fawaz, yUlrich Baumann, zUdeme Ekong, §Bjo¨rn Fischler, jjNedim Hadzic, ôCara L. Mack, #Vale´rie A. McLin, ÃÃJean P. Molleston, yyEzequiel Neimark, zzVicky L. Ng, and §§Saul J. Karpen ABSTRACT Cholestatic jaundice in infancy affects approximately 1 in every 2500 term PREAMBLE infants and is infrequently recognized by primary providers in the setting of holestatic jaundice in infancy is an uncommon but poten- physiologic jaundice. Cholestatic jaundice is always pathologic and indicates tially serious problem that indicates hepatobiliary dysfunc- hepatobiliary dysfunction. Early detection by the primary care physician and tion.C Early detection of cholestatic jaundice by the primary care timely referrals to the pediatric gastroenterologist/hepatologist are important physician and timely, accurate diagnosis by the pediatric gastro- contributors to optimal treatment and prognosis. The most common causes of enterologist are important for successful treatment and an optimal cholestatic jaundice in the first months of life are biliary atresia (25%–40%) prognosis. The Cholestasis Guideline Committee consisted of 11 followed by an expanding list of monogenic disorders (25%), along with many members of 2 professional societies: the North American Society unknown or multifactorial (eg, parenteral nutrition-related) causes, each of for Gastroenterology, Hepatology and Nutrition, and the European which may have time-sensitive and distinct treatment plans. Thus, these Society for Gastroenterology, Hepatology and Nutrition. This guidelines can have an essential role for the evaluation of neonatal cholestasis committee has responded to a need in pediatrics and developed to optimize care. -
Pancreaticobiliary Ductal Union Gut: First Published As 10.1136/Gut.31.10.1144 on 1 October 1990
1144 Gut, 1990, 31, 1144-1149 Pancreaticobiliary ductal union Gut: first published as 10.1136/gut.31.10.1144 on 1 October 1990. Downloaded from S P Misra, M Dwivedi Abstract TABLE ii Length ofthe common channel in normalsubjects in The main pancreatic duct and the common bile various series duct open into the second part of the duo- Authors Mean (mm) Range (mm) denum alone or after joining as a common Misra et al4 4-7 1-018-4 channel. A common channel of >15 mm (an Kimura et al5 4.6 2-10 anomalous pancreaticobiliary duct) is associ- Dowdy et al6 4-4 1-12 ated with congenital cystic dilatation of the common bile duct and carcinoma of the gall bladder. Even a long common channel channel of <3 mm, and 18% had a common (38 mm) is associated with a higher frequency channel of >3 mm. The rhean length was not of carcinoma of the gall bladder. Gail stones mentioned.2 In a necropsy study of 35 infants, smaller than the common channel and a long Miyano et al' noted that the average length of the common channel predispose to gail stone common channel was 1 3 mm. induced acute pancreatitis. Separate openings Kimura et al,' using cineradiography during for the two ductal systems predisposes to ERCP, have shown contractile motility of the development of gall stones and alcohol ductal wall extending well beyond the common induced chronic pancreatitis. The role of channel, towards the liver. The mean (SD) ductal union has also been investigated in length of the contractile segment was 20 5 primary sclerosing cholangitis and biliary (4 6) mm (range 14-31 mm). -
What Is the Normal Size of the Common Bile Duct?
DOI: https://doi.org/10.22516/25007440.136 Original articles What is the normal size of the common bile duct? Martín Alonso Gómez Zuleta, MD,1 Óscar Fernando Ruiz Morales, MD,2 William Otero Regino, MD.3 1 Internist and Gastroenterologist, Professor at the Abstract National University of Colombia. Gastroenterologist at National University of Colombia Hospital in Traditionally, the common bile duct (CBD) has been said to measure up to 6 mm in patients with gallbladders Bogotá, Colombia and up to 8 mm in cholecystectomized patients. However, these recommendations are based on very old 2 Internist and Gastroenterologist at Hospital Nacional studies performed with trans-abdominal ultrasound. Echoendoscopy has greater sensitivity and specificity for Universitario and Kennedy Hospital in Bogotá, Colombia evaluating the bile duct, but studies had not yet been done in our population to evaluate the normal size of 3 Internist and Gastroenterologist, Professor of the CBD by this method. Gastroenterology at the National University of Objective: The objective of this study was to evaluate the size of the CBD in patients with gallbladders and Colombia in Bogotá, Colombia patients without gallbladders. Materials and Methods: This is a prospective descriptive study of patients who underwent echoendoscopy ......................................... at the gastroenterology unit in the El Tunal hospital, Universidad Nacional de Colombia. Patients had been Received: 05-11-15 Accepted: 21-04-17 referred for diagnostic echoendoscopy to evaluate subepithelial lesions in the esophagus and/or stomach. Once the lesion had been evaluated and an echoendoscopic diagnosis had been established, the transducer was advanced to the second duodenal portion to perform bilio-pancreatic echoendoscopy. -
Expression of Specific Hepatocyte and Cholangiocyte Transcription Factors
Laboratory Investigation (2008) 88, 865–872 & 2008 USCAP, Inc All rights reserved 0023-6837/08 $30.00 Expression of specific hepatocyte and cholangiocyte transcription factors in human liver disease and embryonic development Pallavi B Limaye, Gabriela Alarco´n, Andrew L Walls, Michael A Nalesnik, George K Michalopoulos, Anthony J Demetris and Erin R Ochoa Transcription factors are major determinants of cell-specific gene expression in all cell types. Studies in rodent liver have shown that alterations in transcription factor expression determine lineage specification during fetal liver development and signify transdifferentiation of cells of the biliary compartment into ‘oval’ cells and eventually hepatocytes in adult liver. We examined the cellular localization of hepatocyte- or BEC-associated transcription factors in human fetal and adult liver and in diseases in which transdifferentiation between hepatocytes and biliary cells may play a role. In the normal adult human liver, hepatocyte nuclear factor (HNF)4a and HNF6 appeared exclusively in hepatocytes; HNF1b, HNF3a, and HNF3b were observed only in BEC. During fetal development both BEC and hepatocytes expressed HNF3a, HNF3b, and HNF6. HNF1a was expressed only in fetal hepatocytes. We further examined expression of transcription factors in massive hepatic necrosis and in specific types of chronic liver disease. Hepatocyte-associated transcription factors HNF4a and HNF6 also appeared in BEC in massive hepatic necrosis and chronic hepatitis C virus infection. Similarly, HNF3b that is expressed only in BEC in normal adult liver was also observed in hepatocytes in primary biliary cirrhosis and chronic biliary obstruction. These data mimic previous findings in rodents in which hepatocyte-associated transcription factors appear in biliary cells prior to emergence of oval cells, which function as progenitor cells for hepatocytes when the regenerative capacity of the latter is compromised. -
Neoplasms of the Liver
Modern Pathology (2007) 20, S49–S60 & 2007 USCAP, Inc All rights reserved 0893-3952/07 $30.00 www.modernpathology.org Neoplasms of the liver Zachary D Goodman Department of Hepatic and Gastrointestinal Pathology, Armed Forces Institute of Pathology, Washington, DC, USA Primary neoplasms of the liver are composed of cells that resemble the normal constituent cells of the liver. Hepatocellular carcinoma, in which the tumor cells resemble hepatocytes, is the most frequent primary liver tumor, and is highly associated with chronic viral hepatitis and cirrhosis of any cause. Benign tumors, such as hepatocellular adenoma in a noncirrhotic liver or a large, dysplastic nodule in a cirrhotic liver, must be distinguished from well-differentiated hepatocellular carcinoma. Cholangiocarcinoma, a primary adenocarci- noma that arises from a bile duct, is second in frequency. It is associated with inflammatory disorders and malformations of the ducts, but most cases are of unknown etiology. Cholangiocarcinoma resembles adenocarcinomas arising in other tissues, so a definitive diagnosis relies on the exclusion of an extrahepatic primary and distinction from benign biliary lesions. Modern Pathology (2007) 20, S49–S60. doi:10.1038/modpathol.3800682 Keywords: hepatocellular carcinoma; hepatocellular adenoma; dysplastic nodule; cholangiocarcinoma A basic principle of pathology is that a neoplasm more than 3 to 1 (Figure 1). Among primary liver usually differentiates in the manner of cells that are tumors that come to clinical attention, over three- normally present in the tissue in which the fourths are hepatocellular carcinoma (HCC), while neoplasm arises. Thus, primary neoplasms and the second most common primary malignancy, tumor-like lesions that occur in the liver usually cholangiocarcinoma (CC) accounts for 8% (Figure resemble the major constituent cells of the liver, 2). -
Chronic Pancreatitis: Introduction
Chronic Pancreatitis: Introduction Authors: Anthony N. Kalloo, MD; Lynn Norwitz, BS; Charles J. Yeo, MD Chronic pancreatitis is a relatively rare disorder occurring in about 20 per 100,000 population. The disease is progressive with persistent inflammation leading to damage and/or destruction of the pancreas . Endocrine and exocrine functional impairment results from the irreversible pancreatic injury. The pancreas is located deep in the retroperitoneal space of the upper part of the abdomen (Figure 1). It is almost completely covered by the stomach and duodenum . This elongated gland (12–20 cm in the adult) has a lobe-like structure. Variation in shape and exact body location is common. In most people, the larger part of the gland's head is located to the right of the spine or directly over the spinal column and extends to the spleen . The pancreas has both exocrine and endocrine functions. In its exocrine capacity, the acinar cells produce digestive juices, which are secreted into the intestine and are essential in the breakdown and metabolism of proteins, fats and carbohydrates. In its endocrine function capacity, the pancreas also produces insulin and glucagon , which are secreted into the blood to regulate glucose levels. Figure 1. Location of the pancreas in the body. What is Chronic Pancreatitis? Chronic pancreatitis is characterized by inflammatory changes of the pancreas involving some or all of the following: fibrosis, calcification, pancreatic ductal inflammation, and pancreatic stone formation (Figure 2). Although autopsies indicate that there is a 0.5–5% incidence of pancreatitis, the true prevalence is unknown. In recent years, there have been several attempts to classify chronic pancreatitis, but these have met with difficulty for several reasons.