Malignant Carcinoid Syndrome

Malignant Carcinoid Syndrome
Malignant Carcinoid Syndrome

Overview Of Malignant Carcinoid Syndrome

Malignant carcinoid syndrome is a group of symptoms associated with carcinoid tumors. These are tumors of the small intestine, colon, appendix, and bronchial tubes in the lungs.

Commonly Associated With

Flush syndrome; Argentaffinoma syndrome

Causes Of Malignant Carcinoid Syndrome

Carcinoid syndrome is the pattern of symptoms sometimes seen in people with carcinoid tumors. These tumors are rare, and often slow-growing. Most carcinoid tumors are found in the gastrointestinal tract and lungs.

Carcinoid syndrome occurs in very few people with carcinoid tumors 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. The hormones cause the blood vessels to open (dilate). This causes carcinoid syndrome.

Symptoms Of Malignant Carcinoid Syndrome

The malignant carcinoid syndrome is made up of four main symptoms including:

  • Flushing (face, neck, or upper chest), such as widened blood vessels seen on the skin (telangiectasias)
  • Difficulty breathing, such as wheezing
  • Diarrhea
  • Heart problems, such as leaking heart valves, slow heartbeat, low or high blood pressure
  • Symptoms are sometimes brought on by physical exertion, or eating or drinking things such as blue cheese, chocolate, or red wine.

Exams & Tests

Most of these tumors are found when tests or procedures are done for other reasons, such as during abdominal surgery.

If a physical exam is done, the health care provider may find signs of:

  • Heart valve problems, such as the murmur
  • Niacin-deficiency disease (pellagra)

Tests that may be done include:

  • 5-HIAA levels in urine
  • Blood tests (including serotonin and chromogranin blood test)
  • CT and MRI scan of the chest or abdomen
  • Echocardiogram
  • Octreotide radiolabeled scan

Treatment Of Malignant Carcinoid Syndrome

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 either of the following:

Removing areas of the liver that have tumor cells

  • Sending (infusing) medicine directly into the liver to destroy the tumors
  • When the entire tumor can’t be removed, removing large portions of the tumor (“debulking”) can help relieve the symptoms.
  • Octreotide (Sandostatin) or lanreotide (Somatuline) injections are given to people with advanced carcinoid tumors that can’t be removed with surgery.
  • People with carcinoid syndrome should 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 paroxetine (Paxil) and fluoxetine (Prozac), may make symptoms worse by increasing levels of serotonin. However, DO NOT stop taking these medicines unless your provider tells you to do so.