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Encyclopedia > Carcinoid syndrome
Carcinoid syndrome
Classification & external resources
ICD-10 E34.0
ICD-9 259.2
ICD-O: M8240/3-8245
DiseasesDB 2040
MedlinePlus 000347
eMedicine med/271 

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). 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, 15 cases/1,000,000 population, carcinoid tumors account for 75% of GI endocrine tumors. The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following codes are used with International Statistical Classification of Diseases and Related Health Problems. ... // E00-E35 - Endocrine diseases (E00-E07) Disorders of thyroid gland (E00) Congenital iodine-deficiency syndrome (E01) Iodine-deficiency-related thyroid disorders and allied conditions (E02) Subclinical iodine-deficiency hypothyroidism (E03) Other hypothyroidism (E030) Congenital hypothyroidism with diffuse goitre (E031) Congenital hypothyroidism without goitre (E032) Hypothyroidism due to medicaments and other... The International Statistical Classification of Diseases and Related Health Problems (most commonly known by the abbreviation ICD) provides codes to classify diseases and a wide variety of signs, symptoms, abnormal findings, complaints, social circumstances and external causes of injury or disease. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... The International Classification of Diseases for Oncology (ICD-O) is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems for tumor diseases. ... The International Classification of Diseases for Oncology (ICD-O) is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems for tumor diseases. ... The Disease Bold textDatabase is a free website that provides information about the relationships between medical conditions, symptoms, and medications. ... MedlinePlus (medlineplus. ... eMedicine is an online clinical medical knowledge base that was founded in 1996. ... The term symptom (from the Greek meaning chance, mishap or casualty, itself derived from συμπιπτω meaning to fall upon or to happen to) has two similar meanings in the context of physical and mental health: Strictly, a symptom is a sensation or change in health function experienced by a patient. ... Picture of a carcinoid tumour that encroaches into lumen of the small bowel. ... Tumor or tumour literally means swelling, and is sometimes still used with that meaning. ... The gastrointestinal tract (GI tract), also called the digestive tract, alimentary canal, or gut, is the system of organs within multicellular animals that takes in food, digests it to extract energy and nutrients, and expels the remaining waste. ... In human anatomy, the vermiform appendix (or appendix, pl. ... Grays Fig. ... The rectum (from the Latin rectum intestinum, meaning straight intestine) is the final straight portion of the large intestine in some mammals, and the gut in others, terminating in the anus. ... The endocrine system is a control system of ductless endocrine glands that secrete chemical messengers called hormones that circulate within the body via the bloodstream to affect distant organs. ... Norepinephrine A hormone (from Greek όρμή - to set in motion) is a chemical messenger from one cell (or group of cells) to another. ... Red blood cells (erythrocytes) are present in the blood and help carry oxygen to the rest of the cells in the body Blood is a circulating tissue composed of fluid plasma and cells (red blood cells, white blood cells, platelets). ... In biology, an organ (Latin: organum, instrument, tool) is a group of tissues that perform a specific function or group of functions. ...

Contents

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, 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. 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 system do not occur until there are metastases to the liver. Carcinoid tumors arising in the bronchi, because their biologically active products reach the systemic circulation before passing through the liver, may be associated with manifestations of carcinoid syndrome without liver metastases. Serotonin (5-hydroxytryptamine, or 5-HT) is a monoamine neurotransmitter synthesized in serotonergic neurons in the central nervous system (CNS) and enterochromaffin cells in the gastrointestinal tract of animals including humans. ...


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 scans and MRI with detection rates of about 80%. PET scans, which evaluate for increased metabolism of glucose, may also aid in localizing the carcinoid lesion or evaluating for metastases. In general, a diagnosis (plural diagnoses) covers a broad spectrum, or spectra, of testing in some form of analysis; such tests based on some collective reasoning is called the method of diagnostics, leading then to the results of those tests by ideal (ethics) would then be considered a diagnosis, but... This article or section is in need of attention from an expert on the subject. ... Somatostatin is a hormone. ... Somatostatin is a hormone. ... Nuclear medicine is the branch of medicine that uses unsealed radioactive substances in diagnosis and therapy. ... Somatostatin is a hormone. ...


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 localized the tumour. Often lapratomy 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: An antihistamine is a drug which serves to reduce or eliminate effects mediated by histamine, an endogenous chemical mediator released during allergic reactions, through action at the histamine receptor. ...

Surgery Surgery is the medical specialty that treats diseases or injuries by operative manual and instrumental treatment. ... Chemotherapy is the use of chemical substances to treat disease. ...

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 Sandostatin treatment has increased to about 12 years. Prognosis (older Greek πρόγνωσις, modern Greek πρόγνωση - literally fore-knowing, foreseeing) is a medical term denoting the doctors prediction of how a patients disease will progress, and whether there is chance of recovery. ...


Synonyms

Thorson-Bioerck syndrome, argentaffinoma syndrome, Cassidy-Scholte sydrome, flush syndrome Staining is a biochemical technique of adding a class-specific (DNA, proteins, lipids, carbohydrates) dye to a substrate to qualify or quantify the presence of a specific compound. ...


References

  1. "Endocrine Tumors of the GI Tract and Pancreas." Harrison's Manual of Medicine. Eugene Braunwald. 15th edition. New York: McGraw-Hill, 2002. 298-299.
  2. "Malignant jkvgyhj. New York: Lange Medical Books/McGraw-Hill, 2004. 1625.
  3. "Carcinoid Syndrome." Griffith's 5 Minute Clinical Consult. Mark Dambro. Mobile Version 6.0.139. Lippincott, Williams and Wilkins, 2003.
  4. "Current Status of Gastrointestinal Carcinoids." Gastroenterology. Irvin M. Modlin et al. 2005;128;1717-1751.

See also

Enterochromaffin (EC) cells (or Kulchitsky cells) occur in the epithelia lining the lumen of the gastrointestinal tract (GIT; e. ...

External links


  Results from FactBites:
 
Carcinoid syndrome - Wikipedia, the free encyclopedia (510 words)
Carcinoid syndrome refers to the array of symptoms that occur secondary to carcinoid tumors.
Carcinoid tumors produce the vasoactive substance, serotonin; it is commonly but incorrectly thought that serotonin is the cause of the flushing.
Carcinoid tumors arising in the bronchi, because their biologically active products reach the systemic circulation before passing through the liver, may be associated with manifestations of carcinoid syndrome without liver metastases.
  More results at FactBites »

 
 

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