Carcinoid syndrome

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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

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Overview

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.

Clinical presentation

Clinical findings include:^[1]

• Flushing of the skin
• Diarrhea and abdominal cramps
• Right sided heart disease
• 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 valve and pulmonary valve
  • "TIPS" - Tricuspid Insufficiency, Pulmonic Stenosis (fibrosis of tricuspid and pulmonary valves)

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 syndrome 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.

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%. Usually on CT scan, one will note a spider-like/crab like change in the messentery 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 localized 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)

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

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 Carcinoid Syndrome." Current Medical Diagnosis & Treatment. Lawrence M. Tierney, Jr. 43rd edition. 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

• Kulchitsky cells

External links and Patient Resources

• Caring for Carcinoid Foundation
• Merck
• http://www.netumoradvisor.org
• http://www.carcinoid.org

v • d • e Endocrine pathology: endocrine diseases (E00-35, 240-259)
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
Pancreas	Diabetes mellitus (type 1, type 2, coma, angiopathy, ketoacidosis, nephropathy, neuropathy, retinopathy) - Hypoglycemia - Hyperinsulinism - Zollinger-Ellison syndrome - insulin receptor (Rabson-Mendenhall syndrome)
Parathyroid	Hypoparathyroidism (Pseudohypoparathyroidism) - Hyperparathyroidism (Primary, Secondary, Tertiary)
Pituitary	Hyperpituitarism (Acromegaly, Hyperprolactinaemia, SIADH) - Hypopituitarism (Simmonds' disease/Sheehan's syndrome, Kallmann syndrome, Growth hormone deficiency, Diabetes insipidus) - Adiposogenital dystrophy - Empty sella syndrome
Adrenal	Cushing's syndrome (Nelson's syndrome, Pseudo-Cushing's syndrome) - CAH (Lipoid, 3β, 11β, 17α, 21α) - Hyperaldosteronism (Conn syndrome, Bartter syndrome) - Adrenal insufficiency (Addison's disease) - Hypoaldosteronism
Gonads	ovarian dysfunction (Polycystic ovary syndrome, Premature ovarian failure) - testicular dysfunction (5-alpha-reductase deficiency) - testosterone biosynthesis (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

v • d • e WikiDoc Research Resources for Carcinoid syndrome (Click show to right to view)
Articles on Carcinoid syndrome	Most recent articles on Carcinoid syndrome • Most cited articles on Carcinoid syndrome • Review articles on Carcinoid syndrome • Articles on Carcinoid syndrome in N Eng J Med, Lancet, BMJ
Media (Slides, Video, Images, MP3) on Carcinoid syndrome	Powerpoint slides on Carcinoid syndrome • Images of Carcinoid syndrome • Photos of Carcinoid syndrome • Podcasts & MP3s on Carcinoid syndrome • Videos on Carcinoid syndrome
Evidence Based Medicine Regarding Carcinoid syndrome	Cochrane Collaboration on Carcinoid syndrome • Bandolier on Carcinoid syndrome • TRIP on Carcinoid syndrome
Cost Effectiveness of Carcinoid syndrome	Cost Effectiveness of Carcinoid syndrome
Clinical Trials Involving Carcinoid syndrome	Ongoing Trials on Carcinoid syndrome at Clinical Trials.gov • Trial results on Carcinoid syndrome • Clinical Trials on Carcinoid syndrome at Google
Guidelines / Policies / Government Resources (FDA/CDC) Regarding Carcinoid syndrome	US National Guidelines Clearinghouse on Carcinoid syndrome • NICE Guidance on Carcinoid syndrome • NHS PRODIGY Guidance • FDA on Carcinoid syndrome • CDC on Carcinoid syndrome
Textbook Information on Carcinoid syndrome	Books and Textbook Information on Carcinoid syndrome
Pharmacology Resources on Carcinoid syndrome	Dosing of Carcinoid syndrome • Drug interactions with Carcinoid syndrome • Side effects of Carcinoid syndrome • Allergic reactions to Carcinoid syndrome • Overdose information on Carcinoid syndrome • Carcinogenicity information on Carcinoid syndrome • Carcinoid syndrome in pregnancy • Pharmacokinetics of Carcinoid syndrome •
Genetics, Pharmacogenomics, and Proteinomics of Carcinoid syndrome	Genetics of Carcinoid syndrome • Pharmacogenomics of Carcinoid syndrome • Proteomics of Carcinoid syndrome
Newstories on Carcinoid syndrome	Carcinoid syndrome in the news • Be alerted to news on Carcinoid syndrome • News trends on Carcinoid syndrome
Commentary on Carcinoid syndrome	Blogs on Carcinoid syndrome
Patient Resources on Carcinoid syndrome	Patient resources on Carcinoid syndrome • Discussion groups on Carcinoid syndrome • Patient Handouts on Carcinoid syndrome • Directions to Hospitals Treating Carcinoid syndrome • Risk calculators and risk factors for Carcinoid syndrome
Healthcare Provider Resources on Carcinoid syndrome	Symptoms of Carcinoid syndrome • Causes & Risk Factors for Carcinoid syndrome • Diagnostic studies for Carcinoid syndrome • Treatment of Carcinoid syndrome
Continuing Medical Education (CME) Programs on Carcinoid syndrome	CME Programs on Carcinoid syndrome
International Resources on Carcinoid syndrome	Carcinoid syndrome en Espanol • Carcinoid syndrome en Francais
Business Resources on Carcinoid syndrome	Carcinoid syndrome in the Marketplace • Patents on Carcinoid syndrome
Informatics Resources on Carcinoid syndrome	List of terms related to Carcinoid syndrome

de:Flush-Syndrom

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Acknowledgement and Attribution Regarding Sources of Content

Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .