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Johns Hopkins Symptoms and Remedies

Johns Hopkins Medicine

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Carcinoid Tumors and Carcinoid Syndrome

What is it?

A carcinoid tumor is a growth composed of cells with properties of both endocrine and nerve tissues. By far the most common site for carcinoid tumors is the wall of the intestines, but they may occur in other organs, such as the lungs or pancreas. This rare type of tumor can be benign or malignant. Even when malignant, the tumor grows very slowly and thus may never produce any symptoms or affect general health in any way. Occasionally a tumor may cause abdominal pain or intestinal bleeding or grow large enough to cause intestinal obstruction. In about 10 percent of cases, however, the cells of a malignant carcinoid tumor get into the bloodstream, spread to other organs (especially the liver), and multiply. Carcinoid tumors secrete a number of powerful substances (serotonin, histamine, prostaglandins, and several hormones). Consequently, tumors that have spread to the liver produce unpleasant symptoms that characterize what is called carcinoid syndrome. Although carcinoid tumors can be life-threatening, the tumors grow so slowly that the patient may live 10 or 20 years after initial diagnosis. If they are detected before spreading, surgical treatment may allow for full recovery.

What Causes It?

  • The cause of carcinoid tumors is unknown.
  • Risk factors may include obesity, smoking, and excessive alcohol consumption.

Prevention

  • There is currently no known way to prevent carcinoid tumors.

Diagnosis

  • A urine sample is analyzed for the presence of a byproduct of the breakdown of serotonin, the major substance secreted by carcinoid tumors.
  • Abdominal x-rays and possibly a CT (computed tomography) scan of the abdomen will be done.
  • An octreotide (hormone) scan may be performed.
  • Endoscopy or sigmoidoscopy (examination of the bowel using a small, flexible, illuminated scope) is often performed. A biopsy of any suspicious tissue in the colon may be taken during these procedures.
  • Carcinoid tumors may be detected during abdominal surgery for another, unrelated disorder.

How to Treat It

  • If carcinoid tumors are detected before they have spread or in the early stages after spreading, surgery will be performed in an attempt to remove them.
  • Chemotherapy may be used to slow the progress of the carcinoid tumor.
  • If the carcinoid tumor has invaded the liver, treatment is aimed at relieving the symptoms of carcinoid syndrome. Medical treatments may include serotonin-blocking drugs and bronchodilators (to relieve wheezing and shortness of breath).
  • Niacin supplements may be given because the chemical activity of the tumors results in a niacin deficiency that, in fact, causes a number of the symptoms associated with carcinoid syndrome.
  • Injections of octreotide (a synthetic hormone) may be used to decrease the body's secretion of hormonal substances.

When to Call a Doctor

  • The symptoms of carcinoid syndrome warrant a doctor's attention.