Carcinoid syndrome is a rare condition that happens if you have neuroendocrine tumors. Neuroendocrine tumors (NETs) are tumors that start in specialized cells in your neuroendocrine system.
NETs develop when your neuroendocrine cells begin to rapidly divide and multiply, creating tumors that release unusually large amounts of serotonin and other substances. When that happens, you develop carcinoid syndrome symptoms. Sometimes, a carcinoid syndrome diagnosis is the first indication you have a neuroendocrine tumor.
Very rarely, people with carcinoid syndrome have carcinoid crises, a potentially life-threatening condition that can happen if your carcinoid syndrome goes undiagnosed or untreated.
Is carcinoid syndrome common?
No, it’s not. One study suggests 1 in 5 people with neuroendocrine tumors (NETs) have carcinoid syndrome, and NETs affect about 5 in 100,000 people in the United States.
Carcinoid syndrome happens if you have neuroendocrine tumors. This refers to symptoms that develop from tumors in specialized cells in your neuroendocrine system that make and release hormones. While you can have NETs in your gastrointestinal tract, lungs and pancreas, carcinoid syndrome typically happens when NETs in your gastrointestinal tract or lungs flood your system with hormones.
What are the risk factors for carcinoid syndrome?
The most common risk factor is having a neuroendocrine tumor in your gastrointestinal tract or your lungs.
What are the complications of carcinoid syndrome?
Carcinoid syndrome may cause several kinds of complications, including carcinoid crises, which can be life-threatening.
Other carcinoid syndrome complications include:
Carcinoid heart disease: This rare but potentially life-threatening complication happens when neuroendocrine tumors release hormones that affect the linings in your heart chambers, valves and blood vessels. Carcinoid heart disease may lead to heart failure.
Mesenteric fibrosis: NETs may cause inflammation and scarring of your mesentery, which is a fold of tissue that attaches your intestines to the wall of your belly.
Depression: Carcinoid syndrome may affect your body’s ability to process tryptophan, an essential amino acid that helps to make serotonin. Serotonin is a brain chemical that manages central nervous system processes like mood, behavior and sleep.
Carcinoid crisis
Carcinoid crisis is an extremely rare but life-threatening condition. Triggers for this include surgery or other medical procedures or traumatic events like an injury from an accident. Carcinoid crisis symptoms include:
Wide changes in blood pressure, including low blood pressure (hypotension).
Severe flushing.
Shortness of breath (dyspnea).
Confusion.
People with carcinoid syndrome or a neuroendocrine tumor should carry a card listing their medical condition in case they need emergency medical care. Likewise, people with carcinoid syndrome or an NET who are planning to have surgery should make sure their providers know about their condition. Providers can administer medication before and during surgery to reduce carcinoid crisis risk.
Neuroendocrine tumors cause carcinoid syndrome, so treating the tumors also treats the syndrome.
Those treatments include:
Surgery: Surgery to remove or reduce the size of the neuroendocrine tumors.
Somatostatin analogs: Somatostatin analogs are drugs that may stop your body from making too many hormones. This may slow down the growth of the tumor when cancer cells spread to other parts of your body.
Targeted therapy: Targeted therapy is a type of treatment that uses drugs that target certain genes or proteins to kill cancer cells.
Chemotherapy: This treatment uses drugs that destroy cancer cells.
Embolization: Embolization kills tumors by blocking or closing a blood vessel that feeds the tumor
Healthcare providers may use the following tests to diagnose carcinoid syndrome:
Urine test: This test checks on certain hormone levels.
Physical exam and medical history: Many carcinoid syndrome symptoms are similar to other less serious illnesses. Your healthcare provider will ask questions about specific symptoms, how long you’ve had the symptoms and if your symptoms are getting worse.
Blood tests: These tests check on the amounts of substances that your organs and tissues release into your blood. Substance levels that spike or fall might be signs of disease.
Neuroendocrine positron emission tomography (PET) scan: This test tracks neuroendocrine tumors with a dye called Gallium 68 dotatate.
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