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09-SEPTEMBER-2008 02:07:44 - Carcinoid syndrome Carcinoid syndrome Classification and external resources ICD-10 E34.0 ICD-9 259.2 ICD-O: M8240/3-8245 DiseasesDB 2040 MedlinePlus 000347 eMedicine med/271 MeSH D008303 Carcinoid syndrome refers to the array of symptoms that occur secondary to carcinoid tumors. Carcinoid tumors are discrete, yellow, well-circumscribed tumors that can occur anywhere along the gastrointestinal tract GI and in the lung. They most commonly affect the appendix, ileum, and rectum. These tumors are unique in that they are endocrine in nature. They secrete hormones into the blood stream, which then travel to end organs and act upon them via appropriate receptors. Although quite rare with 15 cases/1,000,000 population, carcinoid tumors account for 75% of GI endocrine tumors. Contents 1 Clinical presentation 2 Pathophysiology 3 Diagnosis 4 Localization of tumour 5 Treatment 6 Prognosis 7 Synonyms 8 References 9 See also 10 External links Clinical presentation Clinical findings include:1 Flushing of the skin Diarrhea and abdominal cramps Right sided heart disease - fibrosis of the tricuspid valve Bronchoconstriction Abdominal pain due to desmoplastic reaction of the mesentery In most patients, increased urinary excretion of 5-HIAA 5-hydroxyindoleacetic acid, a degradation product of serotonin Serotonin causes fibrosis of the tricuspid and pulmonary valves. Elevated levels of circulating serotonin have been associated with cardiac failure, due to fibrous deposits on the endocardium. These deposits are thought to be responsible for the fibrous degeneration of the valve apparatus. TIPS - Tricuspid Insufficiency, Pulmonic Stenosis fibrosis of tricuspid and pulmonary valves Please help improve this section by expanding it. Further information might be found on the talk page or at requests for expansion. June 2008 Pathophysiology Carcinoid tumors produce the vasoactive substance, serotonin. It is commonly, but incorrectly, thought that serotonin is the cause of the flushing. The flushing results from secretion of kallikrein, the enzyme that catalyzes the conversion of kininogen to lysyl-bradykinin. The latter is further converted to bradykinin, one of the most powerful vasodilators known. Other components of the carcinoid syndrome are diarrhea probably caused by serotonin, a pellagra-like syndrome probably caused by diversion of large amounts of tryptophan from synthesis of the vitamin B3, Niacin, to the synthesis of 5-hydroxyindoles including serotonin, fibrotic lesions of the endocardium, particularly on the right side of the heart resulting in insufficiency of the tricuspid valve and, less frequently, the pulmonary valve and, uncommonly, bronchoconstriction. The pathogenesis of the cardiac lesions and the bronchoconstriction is unknown, but the former probably involves activation of serotonin 5-HT2B receptors by serotonin. When the primary tumor is in the gastrointestinal tract, as it is in the great majority of cases, the serotonin and kallikrein are inactivated in the liver; manifestations of carcinoid syndrome do not occur until there are metastases to the liver. Carcinoid tumors arising in the bronchi may be associated with manifestations of carcinoid syndrome without liver metastases because their biologically active products reach the systemic circulation before passing through the liver and being metabolized. Diagnosis With a certain degree of clinical suspicion, diagnosis is made primarily by measuring plasma levels of the secreted glycoprotein Chromogranin A, supported by measuring the 24 hour urine levels of 5-HIAA 5-hydroxyindoleacetic acid, a breakdown product of serotonin. Patients with carcinoid syndrome usually excrete 25 mg of 5-HIAA per day. For localization of both primary lesions and metastasis, the initial imaging method is Octreoscan, where 111Indium labelled somatostatin analogues octreotide are used in scintigraphy for detecting tumors expressing somatostatin receptors. Median detection rates with octreoscan are about 89%, in contrast to other imaging techniques such as CT scan and MRI with detection rates of about 80%. Usually on CT scan, one will note a spider-like/crab like change in the mesentery due to the fibrosis from the release of serotonin. PET scans, which evaluate for increased metabolism of glucose, may also aid in localizing the carcinoid lesion or evaluating for metastases. Localization of tumour Tumour localization may be extremely difficult. Barium swallow and follow-up examination of the intestine may occasionally show the tumour. Capsule video endoscopy has recently been used to localize the tumour. Often laparotomy is the definitive way to localize the tumour. Treatment For symptomatic relief of carcinoid sydrome: Octreotide somatostatin analogue- neutralizes serotonin and decreases urinary 5-HIAA Methysergide maleate antiserotonin agent but not used because of serious side effect of retroperitoneal fibrosis Cyproheptadine antihistamine Alternative treatment for qualifying candidates: Surgical resection of tumor and chemotherapy 5-FU and doxorubicin Niacin could possibly reverse carcinoid cancer. Prognosis Prognosis varies from individual to individual. It ranges from a 95% 5 year survival for localized disease to a 20% 5 year survival for those with liver metastases. The average survival time from the start of octreotide treatment has increased to about 12 years. Synonyms Thorson-Bioerck syndrome Argentaffinoma syndrome Cassidy-Scholte sydrome Flush syndrome References ^ E.Goljan, Pathology, 2nd ed Mosby Elsevier, Rapid Review series. Endocrine Tumors of the GI Tract and Pancreas. Harrison's Manual of Medicine. Eugene Braunwald. 15th ion. New York: McGraw-Hill, 2002. 298-299. Papadakis, Maxine A.; Tierney, Lawrence M.; McPhee, Stephen J. 2004. Malignant Carcinoid Syndrome, Current medical diagnosis treatment 2004, 43rd ed., Stamford, Conn: Lange Medical Books/McGraw Hill, 1625. ISBN 0-07-141743-5. Carcinoid Syndrome. Griffith's 5 Minute Clinical Consult. Mark Dambro. Mobile Version 6.0.139. Lippincott, Williams and Wilkins, 2003. Modlin IM, Kidd M, Latich I, Zikusoka MN, Shapiro MD 2005. Current status of gastrointestinal carcinoids. Gastroenterology 128 6: 1717-51. PMID 15887161. See also Kulchitsky cells External links Caring for Carcinoid Foundation Carcinoid Syndrome Merck http://www.carcinoid.org v d e Endocrine pathology: endocrine diseases E00-35, 240-259 Pancreas/ glucose metabolism Diabetes mellitus types: type 1, type 2, MODY, complications: coma, angiopathy, ketoacidosis, nephropathy, neuropathy, retinopathy Hypoglycemia - Hyperinsulinism - Zollinger-Ellison syndrome - insulin receptor Rabson-Mendenhall syndrome - Insulin resistance Hypothalamic/ pituitary axes Pituitary Hyperpituitarism Acromegaly, Hyperprolactinaemia, SIADH Hypopituitarism Sheehan's syndrome, Kallmann syndrome, Growth hormone deficiency, Diabetes insipidus Adiposogenital dystrophy - Empty sella syndrome - Pituitary apoplexy - ACTH deficiency Thyroid Hypothyroidism Iodine deficiency, Cretinism, Congenital hypothyroidism, Goitre, Myxedema Hyperthyroidism Graves disease, Toxic multinodular goitre, Teratoma with thyroid tissue or Struma ovarii Thyroiditis De Quervain's thyroiditis, Hashimoto's thyroiditis, Riedel's thyroiditis Euthyroid sick syndrome - Thyroid hormone resistance - Thyroid nodule Parathyroid Hypoparathyroidism Pseudohypoparathyroidism - Hyperparathyroidism Primary, Secondary, Tertiary Adrenal Adrenocortical hyperfunction: Cushing's syndrome Nelson's syndrome, Pseudo-Cushing's syndrome - Hyperaldosteronism Conn syndrome, Bartter syndrome CAH Lipoid, 3β, 11β, 17α, 21α Adrenal insufficiency Addison's disease, Waterhouse-Friderichsen syndrome - Hypoaldosteronism Gonads ovarian Polycystic ovary syndrome, Premature ovarian failure testicular 5-alpha-reductase deficiency, 17-beta-hydroxysteroid dehydrogenase deficiency general Hypogonadism, Delayed puberty, Precocious puberty Other Androgen insensitivity syndrome - Autoimmune polyendocrine syndrome - Carcinoid syndrome - Gigantism - Short stature Laron syndrome, Psychogenic dwarfism - Multiple endocrine neoplasia 1, 2 - Progeria - Woodhouse-Sakati syndrome - thymus Abscess of thymus, Thymus hyperplasia see also congenital, neoplasia Retrieved from http://en..org/wiki/Carcinoid_syndrome Categories: Gastroenterology | Endocrinology | CardiologyHidden categories: Articles to be expanded since June 2008 | All articles to be expanded Views Article Discussion this page History Personal tools Log in / create account Navigation Main page Contents Featured content Current events Random article Search Go Search Interaction Community portal Recent changes Contact Donate to Help Toolbox What links here Related changes Upload file Special pages Printable version Permanent link Cite this page Languages Deutsch This page was last modified on 28 August 2008, at 20:0
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