Carcinoid syndrome natural history, complications and prognosis: Difference between revisions
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==Overview== | ==Overview== | ||
If left untreated, patients with carcinoid syndrome may progress to develop [[flushing]], [[diarrhea]], and carcinoid heart disease (valvular heart disease and cardiac dysrythmias). Common complications of carcinoid tumor include increased risk of falls and injury (from [[hypotension]]), [[obstruction|bowel obstruction]], [[gastrointestinal bleeding]], [[heart failure|right-sided heart failure]], and fibrosis of the tricuspid valve and pulmonary valve, and rarely the [[mitral valve]] in cases with left sided involvement. Prognosis is generally good and the 5-year survival rate of patients with carcinoid syndrome is approximately 69.7%. | If left untreated, patients with [[Carcinoid Syndrome|carcinoid syndrome]] may progress to develop [[flushing]], [[diarrhea]], and [[Carcinoid|carcinoid heart disease]] ([[valvular heart disease]] and [[Cardiac arrythmia|cardiac dysrythmias]]). Common [[complications]] of [[carcinoid tumor]] include increased risk of [[falls]] and [[injury]] (from [[hypotension]]), [[obstruction|bowel obstruction]], [[gastrointestinal bleeding]], [[heart failure|right-sided heart failure]], and [[fibrosis]] of the [[Tricuspid valves|tricuspid valve]] and [[pulmonary valve]], and rarely the [[mitral valve]] in cases with left sided involvement. [[Prognosis]] is generally good and the 5-year [[Survival rates|survival rate]] of [[patients]] with [[Carcinoid Syndrome|carcinoid syndrome]] is approximately 69.7%. | ||
==Natural History== | ==Natural History== | ||
*If left untreated, patients with carcinoid syndrome may progress to develop [[flushing]], [[diarrhea]], and carcinoid heart disease (valvular heart disease and cardiac dysrythmias):<ref> General Information About Gastrointestinal (GI) Carcinoid Tumors | *If left untreated, [[patients]] with [[Carcinoid Syndrome|carcinoid syndrome]] may progress to develop [[flushing]], [[diarrhea]], and [[Carcinoid disease|carcinoid]] [[Heart diseases|heart disease]] [[Valvular heart disease|(valvular heart disease]] and [[Cardiac arrythmia|cardiac dysrythmias]]):<ref>General Information About Gastrointestinal (GI) Carcinoid Tumors | ||
. National Cancer Institute. http://www.cancer.gov/types/gi-carcinoid-tumors/hp/gi-carcinoid-treatment-pdq#link/_49_toc Accessed on September 24, 2015</ref> | . National Cancer Institute. http://www.cancer.gov/types/gi-carcinoid-tumors/hp/gi-carcinoid-treatment-pdq#link/_49_toc Accessed on September 24, 2015</ref> | ||
**Pathologically, the cardiac valves become thickened because of fibrosis | **[[Pathological|Pathologically]], the [[cardiac]] [[Valves of the heart|valves]] become thickened because of [[fibrosis]] | ||
**[[Tricuspid]] and pulmonic valves are affected to a greater extent than the [[mitral]] and [[aortic valves]] | **[[Tricuspid]] and [[pulmonic valves]] are affected to a greater extent than the [[mitral]] and [[aortic valves]] | ||
*They have a very slow growth rate compared to most malignant tumors | *They have a very slow [[Growth|growth rate]] compared to most [[malignant tumors]] | ||
==Complications== | ==Complications== | ||
Common complications of carcinoid tumor include:<ref> Carcinoid syndrome. U.S. National Library of Medicine. https://www.nlm.nih.gov/medlineplus/ency/article/000347.htm</ref> | Common [[complications]] of [[carcinoid tumor]] include:<ref>Carcinoid syndrome. U.S. National Library of Medicine. https://www.nlm.nih.gov/medlineplus/ency/article/000347.htm</ref> | ||
*Increased risk of falls and injury (from [[hypotension]]) | *Increased risk of [[falls]] and [[injury]] (from [[hypotension]]) | ||
*[[Obstruction|Bowel obstruction]] | *[[Obstruction|Bowel obstruction]] | ||
*[[Gastrointestinal bleeding]] | *[[Gastrointestinal bleeding]] | ||
*Carcinoid heart disease develops in more than one-third of patients with carcinoid syndrome:<ref> General Information About Gastrointestinal (GI) Carcinoid Tumors | *[[Carcinoid Disease|Carcinoid heart disease]] develops in more than one-third of [[patients]] with [[Carcinoid Syndrome|carcinoid syndrome:]]<ref>General Information About Gastrointestinal (GI) Carcinoid Tumors | ||
. National Cancer Institute. http://www.cancer.gov/types/gi-carcinoid-tumors/hp/gi-carcinoid-treatment-pdq#link/_49_toc Accessed on September 24, 2015</ref> | . National Cancer Institute. http://www.cancer.gov/types/gi-carcinoid-tumors/hp/gi-carcinoid-treatment-pdq#link/_49_toc Accessed on September 24, 2015</ref> | ||
*[[Fibrosis]] of the [[Tricuspid valves|tricuspid valve]] and [[pulmonary valve]], rarely the [[mitral valve]] in cases with left sided involvement | |||
::*Tricuspid and pulmonic regurgitation ("TIPS" - [[tricuspid insufficiency|Tricuspid Insufficiency]], [[pulmonary stenosis|Pulmonic Stenosis]]) | ::*[[Tricuspid]] and [[pulmonic regurgitation]] ("TIPS" - [[tricuspid insufficiency|Tricuspid Insufficiency]], [[pulmonary stenosis|Pulmonic Stenosis]]) | ||
::*Pulmonary stenosis | ::*[[Pulmonary stenosis]] | ||
::*Mitral and aortic insufficiency | ::*[[Mitral]] and [[aortic insufficiency]] | ||
::*Cardiac dysrhythmias | ::*[[Cardiac arrhythmias|Cardiac dysrhythmias]] | ||
==Prognosis== | ==Prognosis== | ||
*Prognosis | *Prognosis o[[Carcinoid tumors|f carcinoid tumor]] is generally good and the 5-yea[[Survival rates|r survival rate]] of [[patients]] is approximately:<ref name="pmid15213627">{{cite journal| author=Maggard MA, O'Connell JB, Ko CY| title=Updated population-based review of carcinoid tumors. | journal=Ann Surg | year= 2004 | volume= 240 | issue= 1 | pages= 117-22 | pmid=15213627 | doi= | pmc=PMC1356383 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15213627 }} </ref> | ||
:*Stomach-75.1% | :*[[Stomach]]-75.1% | ||
:*Small Intestine-76.1% | :*[[Intestine|Small Intestine]]-76.1% | ||
:*Appendix-76.3% | :*[[Appendix]]-76.3% | ||
:*Rectum-87.5% | :*[[Rectum]]-87.5% | ||
*Factors that determine the prognosis of patients with gastrointestinal carcinoid tumors include:<ref>Carcinoid tumor. National cancer institute. http://www.cancer.gov/types/gi-carcinoid-tumors/hp/gi-carcinoid-treatment-pdq</ref> | *Factors that determine the prognosis of patients with [[gastrointestinal]] [[carcinoid tumors]] include:<ref>Carcinoid tumor. National cancer institute. http://www.cancer.gov/types/gi-carcinoid-tumors/hp/gi-carcinoid-treatment-pdq</ref> | ||
:*Site of origin | :*Site of [[origin]] | ||
:*Size of | :*Size of th[[Primary tumor|e primary tumor]] | ||
:*Anatomical extent of disease | :*[[Anatomical]] extent of [[disease]] | ||
*Negative prognostic factors include: | *Negative [[Prognostic|prognostic factors]] include: | ||
**Carcinoid heart disease | **[[Carcinoid]] [[heart]] [[disease]] | ||
**High concentrations of the tumor markers - urinary [[5-HIAA]] and plasma [[chromogranin A]] | **High [[concentrations]] of the [[tumor markers]] - [[urinary]] [[5-HIAA]] and [[Plasma (blood)|plasma]] [[chromogranin A]] | ||
**Metastasis to the liver | **[[Metastases|Metastasis]] to the [[liver]] | ||
**Carcinoid tumor in the thymus | **[[Carcinoid tumor]] in the [[thymus]] | ||
**Overexpression of the proliferation antigen Ki-67 | **Overexpression of the [[proliferation]] [[antigen]] Ki-67 | ||
**Mutation in the ''[[p53]]'' gene | **[[Mutations|Mutation]] in the ''[[p53]]'' [[gene]] | ||
==References== | ==References== | ||
Latest revision as of 21:51, 21 April 2019
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2]
Overview
If left untreated, patients with carcinoid syndrome may progress to develop flushing, diarrhea, and carcinoid heart disease (valvular heart disease and cardiac dysrythmias). Common complications of carcinoid tumor include increased risk of falls and injury (from hypotension), bowel obstruction, gastrointestinal bleeding, right-sided heart failure, and fibrosis of the tricuspid valve and pulmonary valve, and rarely the mitral valve in cases with left sided involvement. Prognosis is generally good and the 5-year survival rate of patients with carcinoid syndrome is approximately 69.7%.
Natural History
- If left untreated, patients with carcinoid syndrome may progress to develop flushing, diarrhea, and carcinoid heart disease (valvular heart disease and cardiac dysrythmias):[1]
- Pathologically, the cardiac valves become thickened because of fibrosis
- Tricuspid and pulmonic valves are affected to a greater extent than the mitral and aortic valves
- They have a very slow growth rate compared to most malignant tumors
Complications
Common complications of carcinoid tumor include:[2]
- Increased risk of falls and injury (from hypotension)
- Bowel obstruction
- Gastrointestinal bleeding
- Carcinoid heart disease develops in more than one-third of patients with carcinoid syndrome:[3]
- Fibrosis of the tricuspid valve and pulmonary valve, rarely the mitral valve in cases with left sided involvement
Prognosis
- Prognosis of carcinoid tumor is generally good and the 5-year survival rate of patients is approximately:[4]
- Stomach-75.1%
- Small Intestine-76.1%
- Appendix-76.3%
- Rectum-87.5%
- Factors that determine the prognosis of patients with gastrointestinal carcinoid tumors include:[5]
- Site of origin
- Size of the primary tumor
- Anatomical extent of disease
- Negative prognostic factors include:
- Carcinoid heart disease
- High concentrations of the tumor markers - urinary 5-HIAA and plasma chromogranin A
- Metastasis to the liver
- Carcinoid tumor in the thymus
- Overexpression of the proliferation antigen Ki-67
- Mutation in the p53 gene
References
- ↑ General Information About Gastrointestinal (GI) Carcinoid Tumors . National Cancer Institute. http://www.cancer.gov/types/gi-carcinoid-tumors/hp/gi-carcinoid-treatment-pdq#link/_49_toc Accessed on September 24, 2015
- ↑ Carcinoid syndrome. U.S. National Library of Medicine. https://www.nlm.nih.gov/medlineplus/ency/article/000347.htm
- ↑ General Information About Gastrointestinal (GI) Carcinoid Tumors . National Cancer Institute. http://www.cancer.gov/types/gi-carcinoid-tumors/hp/gi-carcinoid-treatment-pdq#link/_49_toc Accessed on September 24, 2015
- ↑ Maggard MA, O'Connell JB, Ko CY (2004). "Updated population-based review of carcinoid tumors". Ann Surg. 240 (1): 117–22. PMC 1356383. PMID 15213627.
- ↑ Carcinoid tumor. National cancer institute. http://www.cancer.gov/types/gi-carcinoid-tumors/hp/gi-carcinoid-treatment-pdq