Malignant Carcinoid Syndrome

  Malignant Carcinoid Syndrome

Overview

Carcinoid syndrome occurs when a rare cancerous tumor called a carcinoid tumor secretes certain chemicals into your bloodstream, causing a variety of signs and symptoms. A carcinoid tumor, which is a type of neuroendocrine tumor, occurs most often in the gastrointestinal tract or the lungs.

Carcinoid syndrome typically occurs in people who have carcinoid tumors that are advanced. Treatment for carcinoid syndrome usually involves treating the cancer. However, because most carcinoid tumors don't cause carcinoid syndrome until they're advanced, a cure may not be possible. Medications may be recommended to relieve your carcinoid syndrome symptoms and make you more comfortable.

Symptoms

The signs and symptoms of carcinoid syndrome depend on which chemicals the carcinoid tumor secretes into your bloodstream.

The most common signs and symptoms include:

Skin flushing. The skin on your face and upper chest feels hot and changes color — ranging from pink to purple. Flushing episodes may last from a few minutes to a few hours or longer.

Flushing may happen for no obvious reason, though sometimes it can be triggered by stress, exercise or drinking alcohol.

Facial skin lesions. Purplish areas of spiderlike veins may appear on your nose and upper lip.

Diarrhea. Frequent, watery stools sometimes accompanied by abdominal cramps may occur in people who have carcinoid syndrome.

Difficulty breathing. Asthma-like signs and symptoms, such as wheezing and shortness of breath, may occur at the same time you experience skin flushing.

Rapid heartbeat. Periods of a fast heart rate could be a sign of carcinoid syndrome.

When to see a doctor

Make an appointment with your doctor if you have signs and symptoms that concern you.

Causes

Carcinoid syndrome is caused by a carcinoid tumor that secretes serotonin or other chemicals into your bloodstream. Carcinoid tumors occur most often in the gastrointestinal tract, including your stomach, small intestine, appendix, colon and rectum.

Only a small percentage of carcinoid tumors secrete the chemicals that cause carcinoid syndrome. When these tumors do secrete the chemicals, the liver normally neutralizes the chemicals before they have a chance to travel through your body and cause symptoms.

However, when an advanced tumor spreads (metastasizes) to the liver itself, it may secrete chemicals that aren't neutralized before reaching the bloodstream. Most people who experience carcinoid syndrome have an advanced cancer that has spread to the liver.

Some carcinoid tumors don't have to be advanced to cause carcinoid syndrome. For instance, carcinoid lung tumors that secrete chemicals into the blood do so farther upstream from the liver, which then cannot process and eliminate the chemicals.

Carcinoid tumors in the intestine, on the other hand, secrete the chemicals into blood that must first pass through the liver before reaching the rest of the body. The liver usually neutralizes the chemicals before they can affect the rest of the body.

What causes carcinoid tumors is unclear.

Complications

Having carcinoid syndrome can cause the following complications:

Carcinoid heart disease. Some people with carcinoid syndrome develop carcinoid heart disease. Carcinoid syndrome causes problems with the heart valves, making it difficult for them to function properly. As a result, the heart valves may leak.

Signs and symptoms of carcinoid heart disease include fatigue and shortness of breath. Carcinoid heart disease can eventually lead to heart failure. Surgical repair of damaged heart valves may be an option.

Carcinoid crisis. Carcinoid crisis causes a severe episode of flushing, low blood pressure, confusion and breathing difficulty. Carcinoid crisis can occur in people with carcinoid tumors when they are exposed to certain triggers, including anesthetic used during surgery. Carcinoid crisis can be fatal. Your doctor may give you medications before surgery to reduce the risk of carcinoid crisis.

Diagnosis

Your doctor will assess your signs and symptoms to rule out other causes of skin flushing and diarrhea. If no other causes are found, your doctor may suspect carcinoid syndrome.

To confirm a diagnosis, your doctor may recommend further tests, including:

Urine test. Your urine may contain a substance made when your body breaks down serotonin. An excess amount of this substance could indicate that your body is processing extra serotonin, the chemical most commonly excreted by carcinoid tumors.

Blood test. Your blood may contain high levels of certain substances that are released by some carcinoid tumors.

Imaging tests. Imaging tests may be used to locate the primary carcinoid tumor and determine whether it has spread. Your doctor may start with a computed tomography (CT) scan of your abdomen, because most carcinoid tumors are found in the gastrointestinal tract. Other scans, such as Magnetic resonance imaging (MRI) or nuclear medicine scans, may be helpful in certain situations.

A scope or camera to see inside your body. Your doctor may use a long, thin tube equipped with a lens or camera to examine areas inside your body.

An endoscopy, which involves passing a scope down your throat, may help your doctor see inside your gastrointestinal tract. A bronchoscopy, which uses a scope passed down your throat and into your lungs, can help find lung carcinoid tumors. Passing a scope through your rectum (colonoscopy) can help diagnose rectal carcinoid tumors.

Removing tissue for laboratory testing. A sample of tissue from the tumor (biopsy) may be collected to confirm your diagnosis. What type of biopsy you'll undergo depends on where your tumor is located.

Treatment

Treating carcinoid syndrome involves treating your cancer and may also involve using medications to control your specific signs and symptoms.

Treatments may include:

Surgery. Surgery to remove your cancer or most of your cancer may be an option.

Medications to block cancer cells from secreting chemicals. Injections of the medications octreotide (Sandostatin) and lanreotide (Somatuline Depot) may reduce the signs and symptoms of carcinoid syndrome, including skin flushing and diarrhea. A drug called telotristat (Xermelo) can be combined with these drugs to control diarrhea caused by carcinoid syndrome.

Drugs that deliver radiation directly to the cancer cells. Peptide receptor radionuclide therapy (PRRT) combines a drug that seeks out cancer cells with a radioactive substance that kills them. In peptide receptor radionuclide therapy (PRRT) for carcinoid tumors, the drug is injected into your body, where it travels to the cancer cells, binds to the cells and delivers the radiation directly to them. This therapy is used in people who have advanced cancer that hasn't responded to other treatments.

Stopping blood supply to liver tumors. In a procedure called hepatic artery embolization, a doctor inserts a catheter through a needle near your groin and threads it up to the main artery that carries blood to your liver (hepatic artery). The doctor injects particles designed to clog the hepatic artery, cutting off the blood supply to cancer cells that have spread to the liver. The healthy liver cells survive by relying on blood from other blood vessels.

Killing cancer cells in the liver with heat or cold. Radiofrequency ablation delivers heat through a needle to the cancer cells in the liver, causing the cells to die. Cryotherapy is similar, but it works by freezing the tumor.

Chemotherapy. Chemotherapy uses strong drugs to kill cancer cells. Chemotherapy drugs can be given through a vein (intravenously) or in pill form, or both methods can be used.

TYPE OF DOCTOR AND DEPARTMENT: Oncology (Medical) SPECIALIST CAN DIAGNOSE THIS DISEASE.