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Carcinoid Syndrome




Introduction

Carcinoid Syndrome occurs when a rare cancerous tumor called a carcinoid tumor secretes serotonin and other chemicals into your bloodstream, causing a variety of signs and symptoms. Carcinoid tumors occur most commonly in the gastrointestinal tract, including the stomach, small intestine, appendix, colon and rectum, or in the lungs. In rare circumstances, carcinoid tumors can develop in the ovaries.

Because carcinoid tumors generally grow slowly, they typically don't cause signs and symptoms until quite advanced. You might discover you have carcinoid cancer through a test for an unrelated disease or condition.

Treatment for carcinoid syndrome usually involves treating your cancer. However, because most carcinoid tumors don't cause carcinoid syndrome until they're very advanced, a cure may not be possible. In those cases, medications may relieve the signs and symptoms of carcinoid syndrome and make you more comfortable.

Signs and symptoms

The signs and symptoms you have will depend on what chemicals your carcinoid tumor secretes into your bloodstream. The most common signs and symptoms include:
  • Skin flushing. The skin on your face and upper chest changes color — ranging from pink to red to purple. Flushing episodes last 20 to 30 seconds and make your skin feel hot. Flushing may happen for no obvious reason, though sometimes it can be provoked by eating or drinking alcohol.
  • Facial skin lesions. Purplish areas of spider-like veins may appear on the noses and upper lips of people who've had carcinoid syndrome for many years.
  • Diarrhea. Frequent, watery stools accompanied by painful abdominal cramps may signal 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.
Causes

Carcinoid syndrome is caused by a carcinoid tumor that secretes chemicals into your bloodstream. A small percentage of carcinoid tumors secrete chemicals. Some carcinoid tumors secrete chemicals, but your liver effectively processes the chemicals before they have a chance to travel through your body and cause signs and symptoms. Most people who experience carcinoid syndrome have an advanced tumor that has spread to the liver, where tumors have a better chance of secreting chemicals into the bloodstream.

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 much farther upstream — not directly into the liver, where the chemicals are processed and weakened. Carcinoid tumors in the intestine, on the other hand, secrete their 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.

Treatment

Treating carcinoid syndrome involves treating your cancer. Surgery to remove your cancer or most of your cancer may be an option. If surgery isn't an option because your cancer is too widespread, your doctor may recommend treatment to shrink your tumors. This may reduce the signs and symptoms of carcinoid syndrome.

Treatments could include:
  • Octreotide (Sandostatin). Monthly octreotide injections may slow the rate of growth of your carcinoid tumor and reduce the signs and symptoms of carcinoid syndrome. Octreotide controls flushing and diarrhea in most people with carcinoid syndrome. Side effects of octreotide include abdominal pain and bloating, diarrhea and nausea, though these symptoms may subside with time. Some people can't tolerate the side effects of octreotide and must stop taking the drug.
  • Stopping blood supply to the tumor. 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 releases particles to clog the hepatic artery, cutting off the blood supply to any cancer cells that have spread to the liver. The healthy liver cells survive by relying on blood from other blood vessels. Hepatic artery embolization can be risky, especially in people with liver disease, and the procedure is typically only performed in specialized medical centers. Discuss the benefits and risks with your doctor.
  • Killing cancer cells with heat. Radiofrequency ablation delivers heat through a needle to the cancer cells in the liver, causing the cells to die. Radiofrequency ablation might be an option if you have a small number of liver metastases that are small in size. Radiofrequency ablation is generally safe, though there is a small risk of blood loss and infection.
  • Chemotherapy. Chemotherapy drugs may shrink carcinoid tumors. What side effects you may experience will depend on which chemotherapy drugs you receive. Discuss your particular chemotherapy regimen with your doctor.
Medications to control your specific signs and symptoms also may be available. Your doctor may try various combinations of medications to treat your signs and symptoms based on the specific characteristics of your cancer.

The prognosis for people living with carcinoid cancer varies widely, depending on the extent of tumor spread and whether carcinoid syndrome has developed. As cancer research continues, doctors are finding new ways to treat advanced cancers, which may improve survival.



Information obtained from National Institute of Health
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