Carcinoid syndrome (patient information)

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 * Associate Editor(s)-In-Chief: Jinhui Wu, M.D. Ujjwal Rastogi, MBBS [mailto:urastogi@perfuse.org]

Overview
Carcinoid syndrome is a group of symptoms associated with carcinoid tumors -- tumors of the small intestine, colon, appendix, and bronchial tubes in the lungs.

What are the symptoms of Carcinoid syndrome?
Most carcinoid tumors have no symptoms. They only produce the syndrome about 10% of the time. When symptoms do occur, they may include: Sometimes symptoms are brought on by physical exertion, or eating or drinking things such as blue cheeses, chocolate, or red wine.
 * Abdominal pain that comes and goes
 * Bright red flushing of the face
 * Diarrhea (may be explosive and severe)
 * Heart palpitations
 * Hypotension
 * Wheezing

What causes Carcinoid syndrome?
Carcinoid syndrome is the pattern of symptoms sometimes seen in people with carcinoid tumors. These tumors are rare, and often slow growing. Carcinoid syndrome occurs only 10% of the time, usually after the tumor has spread to the liver or lung. These tumors release too much of the hormone serotonin, as well as several other chemicals that cause the blood vessels to open (dilate). More carcinoid tumors are being diagnosed today than in the past.

Who is at highest risk?
Carcinoid tumors patients are at risk of carcinoid syndrome. Such patients are more likely to experience this syndrome if they have a gastrointestinal carcinoid tumor, especially if it has metastasized i.e spread to the liver.

When to seek urgent medical care?
Call for an appointment with your health care provider if you have symptoms of carcinoid syndrome.

Diagnosis
Most of these tumors are found during abdominal surgery. A physical examination may show: Tests may include:
 * Heart valve lesions
 * Signs of niacin-deficiency disease (pellagra)
 * 5-HIAA levels in urine
 * Blood tests (including serotonin blood test)
 * CT and MRI scan
 * OctreoScan (to identify most carcinoids and other neuroendocrine tumors)

Treatment options
Surgery to remove the tumor is usually the first treatment. It can permanently cure the condition if the tumor is completely removed. If the tumor has spread to the liver, treatment involves destroying the local blood supply or giving chemotherapy directly into the liver. When the entire tumor cannot be removed, removing large portions of the tumor ("debulking") can help relieve the symptoms. Sandostatin (octreotide) injections are sometimes given to people with advanced carcinoid tumors that cannot be removed with surgery. This drug can block and reverse tumor growth. Interferon is often given with octreotide to help stop tumor growth. A serotonin antagonist may be prescribed to control diarrhea and poor absorption of nutrients from the intestines (malabsorption). One of several combinations of chemotherapy may be given through a vein (IV) or by mouth. If one combination does not work, another combination may be effective. About one-third of patients benefit from chemotherapy. Avoid alcohol, large meals, and foods high in tyramine (aged cheeses, avocado, many processed foods), because they may trigger symptoms. Some common medicines, like selective serotonin reuptake inhibitors (SSRIs, such as Paxil, Serzone, and Prozac) may make symptoms worse by increasing levels of serotonin. However, do not stop taking these medicines unless your doctor tells you to do so.

Where to find medical care for Carcinoid syndrome?
Directions to Hospitals Treating Carcinoid syndrome

What to expect (Outlook/Prognosis)?
The outlook in patients with carcinoid syndrome is different from the outlook in patients who have carcinoid tumors without the syndrome. In people with the syndrome, the tumor has usually spread to the liver, which lowers the survival rate. People with carcinoid syndrome are also more likely to have a separate cancer (second primary tumor) at the same time. The outlook is more favorable thanks to new treatment methods, such as Sandostatin.

Possible complications

 * Increased risk of falls and injury (from hypotension)
 * Bowel obstruction (from tumor)
 * Gastrointestinal bleeding
 * Right-sided heart failure