Skip to content
My WebMD Sign In, Sign Up

Cancer Health Center

Font Size

Gastrointestinal Carcinoid Tumors Treatment (PDQ®): Treatment - Health Professional Information [NCI] - General Information About Gastrointestinal Carcinoid Tumors

continued...

Carcinoid heart disease develops in more than one-third of patients with carcinoid syndrome. Pathologically, the cardiac valves become thickened because of fibrosis, and the tricuspid and pulmonic valves are affected to a greater extent than the mitral and aortic valves. Symptoms include:[22]

  • Tricuspid and pulmonic regurgitation.
  • Pulmonary stenosis.
  • Mitral and aortic insufficiency.
  • Cardiac dysrhythmias.

Severe carcinoid heart disease is associated with reduced survival. (Refer to the Prognostic Factors section of this summary for more information.)

Site-Specific Clinical Features

The clinical features of GI carcinoids vary according to anatomical location and cell type.[5,12,24] Most carcinoids in the GI tract are located within 3 feet (~90 cm) of the ileocecal valve, with 50% found in the appendix.[25] They are often detected fortuitously during surgery for another GI disorder or during emergency surgery for appendicitis, GI bleeding, or perforation.[26]

Gastric carcinoids

Most gastric carcinoids are enterochromaffin-like (ECL)-cell carcinoids; rarely, other types may occur in the stomach. (Refer to Table 1 in the Cellular and Pathologic Classification of Gastrointestinal Carcinoid Tumors section of this summary for more information.)

Type I ECL-cell gastric carcinoids, the most common type, typically do not have clinical symptoms. They are often discovered during endoscopy for reflux, anemia, or other reasons; and are typically multifocal. Occurring most commonly in women (female-to-male ratio, 2.5:1) at a mean age of 63 years, achlorhydria may be present, and hypergastrinemia or evidence of antral G-cell hyperplasia is usually found.[5,24,27] These tumors are gastrin-driven and arise in a background of chronic atrophic gastritis of the corpus, usually because of autoimmune pernicious anemia but sometimes caused by Helicobacter pylori infection.[9]

Type II ECL-cell carcinoids, the least common type of gastric carcinoids, occur at a mean age of 50 years with no gender predilection. The hypergastrinemia associated with MEN1-Zollinger-Ellison syndrome (ZES) is thought to promote the ECL-cell hyperplasia that leads to type II tumors.[27,28]

Type I and type II ECL-cell gastric carcinoids have been reported to metastasize in fewer than 10% of cases.[27,29] Type III gastric ECL-cell carcinoids, the second most common type of gastric carcinoid, occur mostly in men (male-to-female ratio, 2.8:1) at a mean age of 55 years.[27] There are no neuroendocrine manifestations, and patients typically present with signs and symptoms related to an aggressive tumor.[5,30]

Duodenal carcinoids

Comprising only 2% to 3% of GI NETs and discovered incidentally or because of symptoms from hormonal or peptide production, duodenal carcinoids may also arise in the periampullary region, obstruct the ampulla of Vater, and produce jaundice.[3,5,31] The age at presentation varies widely (range, 19–90 years; mean age, 53 years).[15,32]

1|2|3|4|5|6|7|8

WebMD Public Information from the National Cancer Institute

Last Updated: February 25, 2014
This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.
Next Article:

Today on WebMD

Building a Support System
Blog
cancer fighting foods
SLIDESHOW
 
precancerous lesions slideshow
SLIDESHOW
quit smoking tips
SLIDESHOW
 
Jennifer Goodman Linn self-portrait
Blog
what is your cancer risk
HEALTH CHECK
 
colorectal cancer treatment advances
Video
breast cancer overview slideshow
SLIDESHOW
 
prostate cancer overview
SLIDESHOW
lung cancer overview slideshow
SLIDESHOW
 
ovarian cancer overview slideshow
SLIDESHOW
Actor Michael Douglas
Article