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Carcinoid) Tumours Gastroenteropancreatic
Downloaded from gut.bmjjournals.com on 8 September 2005 Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours J K Ramage, A H G Davies, J Ardill, N Bax, M Caplin, A Grossman, R Hawkins, A M McNicol, N Reed, R Sutton, R Thakker, S Aylwin, D Breen, K Britton, K Buchanan, P Corrie, A Gillams, V Lewington, D McCance, K Meeran, A Watkinson and on behalf of UKNETwork for neuroendocrine tumours Gut 2005;54;1-16 doi:10.1136/gut.2004.053314 Updated information and services can be found at: http://gut.bmjjournals.com/cgi/content/full/54/suppl_4/iv1 These include: References This article cites 201 articles, 41 of which can be accessed free at: http://gut.bmjjournals.com/cgi/content/full/54/suppl_4/iv1#BIBL Rapid responses You can respond to this article at: http://gut.bmjjournals.com/cgi/eletter-submit/54/suppl_4/iv1 Email alerting Receive free email alerts when new articles cite this article - sign up in the box at the service top right corner of the article Topic collections Articles on similar topics can be found in the following collections Stomach and duodenum (510 articles) Pancreas and biliary tract (332 articles) Guidelines (374 articles) Cancer: gastroenterological (1043 articles) Liver, including hepatitis (800 articles) Notes To order reprints of this article go to: http://www.bmjjournals.com/cgi/reprintform To subscribe to Gut go to: http://www.bmjjournals.com/subscriptions/ Downloaded from gut.bmjjournals.com on 8 September 2005 iv1 GUIDELINES Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours J K Ramage*, A H G Davies*, J ArdillÀ, N BaxÀ, M CaplinÀ, A GrossmanÀ, R HawkinsÀ, A M McNicolÀ, N ReedÀ, R Sutton`, R ThakkerÀ, S Aylwin`, D Breen`, K Britton`, K Buchanan`, P Corrie`, A Gillams`, V Lewington`, D McCance`, K Meeran`, A Watkinson`, on behalf of UKNETwork for neuroendocrine tumours .............................................................................................................................. -
Carcinoid Case Presentation and Discussion: the American Perspective
Endocrine-Related Cancer (2003) 10 489–496 NEUROENDOCRINE TUMOURS Carcinoid case presentation and discussion: the American perspective R R P Warner Department of Medicine, Gastrointestinal Division, The Mount Sinai School of Medicine, One Gustave L Levy Place, New York, New York 10029, USA (Requests for offprints should be addressed toRRPWarner; Email: rwarner—[email protected]) Abstract The rationale underlying an aggressive approach in the management of some carcinoid patients is explained and illustrated by the presented case of a middle-aged man with advanced classic typical midgut carcinoid. The patient exhibited somatostatin receptor scintigraphy-positive massive liver metastases, carcinoid syndrome, severe tricuspid and pulmonic cardiac valve disease with congestive heart failure, ascites and malnutrition. He had been treated for several years with supportive medications and biotherapy including octreotide and alpha interferon but his tumor eventually progressed and his overall condition was markedly deteriorated when he first sought more aggressive treatment. This consisted of prompt replacement of both tricuspid and pulmonic valves, followed by hepatic artery chemoembolus (HACE) injection and then surgical tumor debulking including excision of the primary tumor in the small intestine. In addition, radiofrequency ablation was utilized to reduce the volume of metastases in the liver. Prophylactic cholecystectomy was also performed and a biopsy of tumor was submitted for cell culture drug resistance testing. This was followed by systemic chemotherapy utilizing the drug (docetaxel) which the in vitro studies suggested as most likely to be effective. His excellent response to this succession of treatments exemplifies the successful application of aggressive sequential multi-modality therapy. Endocrine-Related Cancer (2003) 10 489–496 Introduction and sometimes aggressive treatment (O¨ berg 1998). -
What Is a Gastrointestinal Carcinoid Tumor?
cancer.org | 1.800.227.2345 About Gastrointestinal Carcinoid Tumors Overview and Types If you have been diagnosed with a gastrointestinal carcinoid tumor or are worried about it, you likely have a lot of questions. Learning some basics is a good place to start. ● What Is a Gastrointestinal Carcinoid Tumor? Research and Statistics See the latest estimates for new cases of gastrointestinal carcinoid tumor in the US and what research is currently being done. ● Key Statistics About Gastrointestinal Carcinoid Tumors ● What’s New in Gastrointestinal Carcinoid Tumor Research? What Is a Gastrointestinal Carcinoid Tumor? Gastrointestinal carcinoid tumors are a type of cancer that forms in the lining of the gastrointestinal (GI) tract. Cancer starts when cells begin to grow out of control. To learn more about what cancer is and how it can grow and spread, see What Is Cancer?1 1 ____________________________________________________________________________________American Cancer Society cancer.org | 1.800.227.2345 To understand gastrointestinal carcinoid tumors, it helps to know about the gastrointestinal system, as well as the neuroendocrine system. The gastrointestinal system The gastrointestinal (GI) system, also known as the digestive system, processes food for energy and rids the body of solid waste. After food is chewed and swallowed, it enters the esophagus. This tube carries food through the neck and chest to the stomach. The esophagus joins the stomachjust beneath the diaphragm (the breathing muscle under the lungs). The stomach is a sac that holds food and begins the digestive process by secreting gastric juice. The food and gastric juices are mixed into a thick fluid, which then empties into the small intestine. -
Familial Occurrence of Carcinoid Tumors and Association with Other Malignant Neoplasms1
Vol. 8, 715–719, August 1999 Cancer Epidemiology, Biomarkers & Prevention 715 Familial Occurrence of Carcinoid Tumors and Association with Other Malignant Neoplasms1 Dusica Babovic-Vuksanovic, Costas L. Constantinou, tomies (3). The most frequent sites for carcinoid tumors are the Joseph Rubin, Charles M. Rowland, Daniel J. Schaid, gastrointestinal tract (73–85%) and the bronchopulmonary sys- and Pamela S. Karnes2 tem (10–28.7%). Carcinoids are occasionally found in the Departments of Medical Genetics [D. B-V., P. S. K.] and Medical Oncology larynx, thymus, kidney, ovary, prostate, and skin (4, 5). Ade- [C. L. C., J. R.] and Section of Biostatistics [C. M. R., D. J. S.], Mayo Clinic nocarcinomas and carcinoids are the most common malignan- and Mayo Foundation, Rochester, Minnesota 55905 cies in the small intestine in adults (6, 7). In children, they rank second behind lymphoma among alimentary tract malignancies (8). Carcinoids appear to have increased in incidence during the Abstract past 20 years (5). Carcinoid tumors are generally thought to be sporadic, Carcinoid tumors were originally thought to possess a very except for a small proportion that occur as a part of low metastatic potential. In recent years, their natural history multiple endocrine neoplasia syndromes. Data regarding and malignant potential have become better understood (9). In the familial occurrence of carcinoid as well as its ;40% of patients, metastases are already evident at the time of potential association with other neoplasms are limited. A diagnosis. The overall 5-year survival rate of all carcinoid chart review was conducted on patients indexed for tumors, regardless of site, is ;50% (5). -
Carcinoid Syndrome Caused by a Serotonin-Secreting Pituitary Tumour
L A Lyngga˚ rd and others Carcinoid syndrome from 170:2 K5–K9 Case Report pituitary tumour Carcinoid syndrome caused by a serotonin-secreting pituitary tumour Correspondence ˚ Louise A Lynggard, Eigil Husted Nielsen and Peter Laurberg should be addressed Department of Endocrinology, Aalborg University Hospital, Hobrovej 18-22, DK-9000 Aalborg, Denmark to P Laurberg Email [email protected] Abstract Background: Neuroendocrine tumours are most frequently located in the gastrointestinal organ system or in the lungs, but they may occasionally be found in other organs. Case: We describe a 56-year-old woman suffering from a carcinoid syndrome caused by a large serotonin-secreting pituitary tumour. She had suffered for years from episodes of palpitations, dyspnoea and flushing. Cardiac disease had been suspected, which delayed the diagnosis, until blood tests revealed elevated serotonin and chromogranin A in plasma. The somatostatin receptor (SSR) scintigraphy showed a single-positive focus in the region of the pituitary gland and MRI showed a corresponding intra- and suprasellar heterogeneous mass. After pre-treatment with octreotide leading to symptomatic improvement, the patient underwent trans-cranial surgery with removal of the tumour. This led to a clinical improvement and to a normalisation of SSR scintigraphy, as well as serotonin and chromogranin A levels. Conclusion: To our knowledge, this is the first reported case of a serotonin-secreting tumour with a primary location in the pituitary. European Journal of Endocrinology (2014) 170, K5–K9 European Journal of Endocrinology Introduction The carcinoid syndrome consists of a variety of symptoms neuroendocrine tumour (NET), most often located in the which typically include episodes of dry flushing with or gut or in the lungs. -
Gastric Neuroendocrine Tumors (Carcinoids)
Current Gastroenterology Reports (2019) 21: 13 https://doi.org/10.1007/s11894-019-0684-7 STOMACH AND DUODENUM (J PISEGNA AND J BENHAMMOU, SECTION EDITORS) Gastric Neuroendocrine Tumors (Carcinoids) Craig R. Gluckman1 & David C. Metz1 Published online: 12 March 2019 # Springer Science+Business Media, LLC, part of Springer Nature 2019 Abstract Purpose of Review The diagnosis of gastric neuroendocrine tumors (NETs) is being made with increased frequency likely as a result of more upper endoscopies being done for unrelated reasons. It is therefore vital that gastroenterologists become familiar with the basic work-up and management of patients found to have these tumors. This review describes the classification, pathophysiology, clinical characteristics, and treatment options of the different gastric NETs. Recent Findings In addition to the three traditional subtypes of gastric NETs, additional cases associated with achlorhydria and appropriate hypergastrinemia may exist. The management of gastric NETs between 1 and 2 cm in size remains controversial and needs to be individualized. Summary Gastric NETs are uncommon but are now diagnosed more frequently. This review highlights the role of hypergastrinemia in their development and the controversies around their management. Keywords Neuroendocrine tumor (NET) . Gastric carcinoid . Hypergastrinemia . Netazepide Introduction increased 15-fold from 1973 to 2012 based on the Surveillance, Epidemiology, and End Results (SEER) database, with the most Gastroenteropancreatic neuroendocrine tumors (GEPNETs) recent annual incidence estimated at 0.5 per 100,000 persons [3]. are commonly divided into pancreatic neuroendocrine tumors Gastric NETs are relatively rare lesions representing about 7% of (pNETs) and (luminal) carcinoid tumors with the latter being all carcinoid tumors and less than 1% of all stomach neoplasms further subdivided into foregut, midgut, and hindgut subtypes [4]. -
Carcinoid Syndrome and Perioperative Anesthetic Considerations Kenneth Mancuso MD (Assistant Professor)A, Alan D
Journal of Clinical Anesthesia (2011) 23, 329–341 Review article Carcinoid syndrome and perioperative anesthetic considerations Kenneth Mancuso MD (Assistant Professor)a, Alan D. Kaye MD, PhD (Professor, Chairman)a, J. Philip Boudreaux MD, FACS (Professor)b,c, Charles J. Fox MD (Associate Professor)d,e, Patrick Lang (Medical Student)e, Philip L. Kalarickal MD, MPH (Clinical Assistant Professor)f, Santiago Gomez MD (Clinical Assistant Professor)f, Paul J. Primeaux MD (Clinical Assistant Professor)g,⁎ aDepartment of Anesthesiology, Louisiana State University Health Sciences Center, New Orleans, LA 70112, USA bDivision of Neuroendocrine Surgery and Transplantation, Louisiana State University Health Sciences Center, New Orleans, LA cChairman, Department of Surgery, Ochsner-Kenner Hospital, Kenner, LA 70065, USA dDirector of Perioperative Management, Department of Anesthesiology, Tulane University School of Medicine, New Orleans, LA 70112, USA eTulane University School of Medicine, New Orleans, LA 70112, USA fDepartment of Anesthesiology, Tulane University School of Medicine, New Orleans, LA 70112, USA gResidency Program Director, Department of Anesthesiology, Tulane University School of Medicine, New Orleans, LA 70112, USA Received 10 February 2010; revised 7 December 2010; accepted 8 December 2010 Keywords: Abstract Carcinoid tumors are uncommon, slow-growing neoplasms. These tumors are capable of Carcinoid syndrome; secreting numerous bioactive substances, which results in significant potential challenges in the Octreotide; management of patients afflicted with carcinoid syndrome. Over the past two decades, both surgical and Serotonin medical therapeutic options have broadened, resulting in improved outcomes. The pathophysiology, clinical signs and symptoms, diagnosis, treatment options, and perioperative management, including anesthetic considerations, of carcinoid syndrome are presented. © 2011 Elsevier Inc. -
Clinical Presentation and Diagnosis of Pancreatic Neuroendocrine Tumors
Clinical Presentation and Diagnosis of Pancreatic Neuroendocrine Tumors Carinne W. Anderson, MD*, Joseph J. Bennett, MD KEYWORDS Pancreatic neuroendocrine tumor Nonfunctional pancreatic neuroendocrine tumor Insulinoma Gastrinoma Glucagonoma VIPoma Somatostatinoma KEY POINTS Pancreatic neuroendocrine tumors are a rare group of neoplasms, most of which are nonfunctioning. Functional pancreatic neoplasms secrete hormones that produce unique clinical syndromes. The key management of these rare tumors is to first suspect the diagnosis; to do this, cli- nicians must be familiar with their clinical syndromes. Pancreatic neuroendocrine tumors (PNETs) are a rare group of neoplasms that arise from multipotent stem cells in the pancreatic ductal epithelium. Most PNETs are nonfunctioning, but they can secrete various hormones resulting in unique clinical syn- dromes. Clinicians must be aware of the diverse manifestations of this disease, as the key step to management of these rare tumors is to first suspect the diagnosis. In light of that, this article focuses on the clinical features of different PNETs. Surgical and medical management will not be discussed here, as they are addressed in other arti- cles in this issue. EPIDEMIOLOGY Classification PNETs are classified clinically as nonfunctional or functional, based on the properties of the hormones they secrete and their ability to produce a clinical syndrome. Nonfunctional PNETs (NF-PNETs) do not produce a clinical syndrome simply because they do not secrete hormones or because the hormones that are secreted do not The authors have nothing to disclose. Department of Surgery, Helen F. Graham Cancer Center, 4701 Ogletown-Stanton Road, S-4000, Newark, DE 19713, USA * Corresponding author. E-mail address: [email protected] Surg Oncol Clin N Am 25 (2016) 363–374 http://dx.doi.org/10.1016/j.soc.2015.12.003 surgonc.theclinics.com 1055-3207/16/$ – see front matter Ó 2016 Elsevier Inc. -
Rare Diseases, When Taken Together, Are Are Together, When Taken Diseases, Rare to According at All
2013 MEDICINES IN DEVELOPMENT REPORT Rare Diseases A Report on Orphan Drugs in the Pipeline PRESENTED BY AMERICA’s biopharmACEUTICAL RESEARCH COMPANIES More Than 450 Medicines in Development for Rare Diseases Rare diseases, when taken together, are A major area of this research targets Orphan Drugs in Development* not that rare at all. In fact, according to rare cancers, accounting for more than Application the National Institutes of Health (NIH), one-third of all rare disease medicines in Submitted 30 million Americans have one of the development. Other top research areas Phase III nearly 7,000 diseases that are officially include genetic disorders, neurologi- Phase II deemed “rare” because alone they each cal conditions, infectious diseases and Phase I affect fewer than 200,000 people in autoimmune disorders. the United States. Sometimes, only Despite some recent victories, research a few hundred patients are known to 105 into treatments for rare diseases is a have a particular rare disease. daunting quest. This ongoing innovation Simply receiving a diagnosis of a rare and the hundreds of new medicines in disease often becomes a frustrating development now offer hope that physi- 85 quest, since many doctors may have nev- cians will have new treatment options er before heard of or seen the disease. for patients confronting a rare disease. This is, however, a time of great progress and hope. Biopharmaceutical 65 Contents research is entering an exciting new era Innovative Orphan Drugs with a growing understanding of the in the Pipeline ......................................... 2 human genome. Scientific advances have given researchers new tools to Orphan Drug Approvals ...........................4 explore rare diseases, which are often Challenges in Clinical Trials ......................6 more complex than common diseases. -
Clinical Diagnosis of Nets
The Role of the Gastroenterologist in the Diagnosis and Treatment of NETS David C. Metz, MD Professor of Medicine Perelman School of Medicine at the University of Pennsylvania Neuroendocrine Tumors • Second most prevalent cancer of the GI tract 1 behind colorectal cancer • Over 100,000 patients are living with NETs in the United States • Principles of care are different/unique compared to other solid tumors JC et al. One hundred years after "Carcinoid": epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol 2008;26:3063–72. Incidence of Neuroendocrine Tumors Over Time is Increasing Analysis of SEER database (1973–2004)1 Yao JC et al. One hundred years after "Carcinoid": epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol 2008;26(18):3063–72. Classic vs NET Tumor Size Paradigm Metastases Lymph Nodes Primary Classic solid tumor Neuroendocrine tumor NETs: Three Patterns of Presentation 1. Hormonal syndrome Need to put 2 and 2 together (requires expertise) 2. Tumor symptoms (from growth) Usually present late (with mets) 3. Asymptomatic (incidental finding) Locoregional (resectable) vs. Widespread Early diagnosis has prognostic implications because surgery is the ONLY curative treatment modality Obstacles in the Early Diagnosis of NETs • Rare Diseases – Zebras, needle in haystack • Other more common diseases – Wolf in sheep’s clothing • Presentation varies from case to case • Often asymptomatic initially • Awareness of problem is low • Requires astute physician with a high index of suspicion Delay in Diagnosis of Neuroendocrine Tumors1 • Currently over 90% of NET patients are misdiagnosed and treated for the wrong disease • Average time to correct diagnosis is 5-7 years • IBS and Inflammatory Bowel Disease (IBD) are the two most common misdiagnosed conditions for patients with midgut carcinoid • Diagnosis is usually not made until metastases to liver and carcinoid syndrome symptoms occur 1. -
Serotonin-Secreting Neuroendocrine Tumours of the Pancreas
Journal of Clinical Medicine Article Serotonin-Secreting Neuroendocrine Tumours of the Pancreas Anna Caterina Milanetto 1,* , Matteo Fassan 2 , Alina David 1 and Claudio Pasquali 1 1 Pancreatic and Endocrine Digestive Surgical Unit, Department of Surgery, Oncology and Gastroenterology, University of Padua, via Giustiniani, 2-35128 Padua, Italy; [email protected] (A.D.); [email protected] (C.P.) 2 Surgical Pathology Unit, Department of Medicine, University of Padua, via Giustiniani, 2-35128 Padua, Italy; [email protected] * Correspondence: [email protected]; Tel.: +39-0498-218-831 Received: 3 April 2020; Accepted: 2 May 2020; Published: 6 May 2020 Abstract: Background: Serotonin-secreting pancreatic neuroendocrine tumours (5-HT-secreting pNETs) are very rare, and characterised by high urinary 5-hydroxyindole-acetic acid (5-HIAA) levels (or high serum 5-HT levels). Methods: Patients with 5-HT-secreting pancreatic neoplasms observed in our unit (1986–2015) were included. Diagnosis was based on urinary 5-HIAA or serum 5-HT levels. Results: Seven patients were enrolled (4 M/3 F), with a median age of 64 (range 38–69) years. Two patients had a carcinoid syndrome. Serum 5-HT was elevated in four patients. Urinary 5-HIAA levels were positive in six patients. The median tumour size was 4.0 (range 2.5–10) cm. All patients showed liver metastases at diagnosis. None underwent resective surgery; lymph node/liver biopsies were taken. Six lesions were well-differentiated tumours and one a poorly differentiated carcinoma (Ki67 range 3.4–70%). All but one patient received chemotherapy. Four patients received somatostatin analogues; three patients underwent ablation of liver metastases. -
Gastrointestinal Carcinoid Tumor Risk Factors ● What Causes Gastrointestinal Carcinoid Tumors?
cancer.org | 1.800.227.2345 Gastrointestinal Carcinoid Tumor Causes, Risk Factors, and Prevention Risk Factors A risk factor is anything that affects your chance of getting a disease such as cancer. Learn more about the risk factors for gastrointestinal carcinoid tumors. ● Gastrointestinal Carcinoid Tumor Risk Factors ● What Causes Gastrointestinal Carcinoid Tumors? Prevention At this time, there is no known way to prevent gastrointestinal carcinoid tumors. Since smoking might increase the risk of carcinoid tumors of the small intestine, not starting or quitting smoking may reduce the risk for this disease. 1 ● About Gastrointestinal Carcinoid Tumors ● Causes, Risk Factors, and Prevention 2 ● Early Detection, Diagnosis, and Staging 3 ● Treatment 4 ● After Treatment Gastrointestinal Carcinoid Tumor Risk 1 ____________________________________________________________________________________American Cancer Society cancer.org | 1.800.227.2345 Factors A risk factor is anything that increases your chance of getting a disease such as cancer. Different cancers have different risk factors. Some risk factors, like smoking, can be changed. Others, like a person’s age or family history, can’t be changed. In some cases, there might be a factor that may decrease your risk of developing cancer. That is not considered a risk factor, but you may see them noted clearly on this page as well. But having a risk factor, or even many, does not mean that you will get cancer. And some people who get cancer may not have any known risk factors. Here are some of the risk factors known to increase your risk for GI carcinoid tumors. Genetic syndromes Multiple endocrine neoplasia, type I This is a rare condition caused by inherited defects in the MEN1 gene.