A single copy of this article may be reprinted for personal, noncommercial use only.
Carcinoid syndromeBy Mayo Clinic staff
Original Article: http://www.mayoclinic.com/health/carcinoid-syndrome/DS00690
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. Carcinoid tumors occur most commonly in the gastrointestinal tract or 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. In those cases, medications may relieve your symptoms of carcinoid syndrome and make you more comfortable.
The signs and symptoms of carcinoid syndrome depend on which chemicals your carcinoid tumor secretes into your bloodstream. The most common signs and symptoms of carcinoid syndrome include:
- Skin flushing. The skin on your face and upper chest feels hot and changes color — ranging from pink to red 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 spider-like veins may appear on the 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 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.
Carcinoid syndrome is caused by a carcinoid tumor that secretes serotonin or other chemicals into your bloodstream. Carcinoid tumors occur most commonly in your gastrointestinal tract, including your stomach, small intestine, appendix, colon and rectum, or in your lungs.
Only a small percentage of carcinoid tumors secrete the chemicals that cause carcinoid syndrome. In most cases, the liver effectively 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, these tumors 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 — not directly into the liver, where the chemicals are processed and eliminated. 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.
Having carcinoid syndrome can cause the following complications:
- Carcinoid heart disease. Some people with carcinoid syndrome develop carcinoid heart disease. Carcinoid syndrome causes a thickening of 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 during physical activity. Carcinoid heart disease can eventually lead to heart failure. Your doctor may recommend medications for your heart. Surgical repair of damaged heart valves may be an option in advanced carcinoid heart disease.
- Bowel obstruction. Cancer that spreads to the lymph nodes next to your small intestine can cause narrowing and kinking of your intestine, leading to a bowel obstruction. Signs and symptoms of a bowel obstruction include severe, cramping abdominal pain and vomiting. Surgery may be necessary to relieve the obstruction.
- 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 anesthesia used during surgery. Carcinoid crisis can be fatal. Your doctor may give you medications before surgery to reduce the risk of carcinoid crisis.
Preparing for your appointment
You may start by seeing your family doctor or a general practitioner if you have signs and symptoms of carcinoid syndrome. Based on what your doctor finds, you may be referred to a doctor who specializes in treating cancer (oncologist), disorders of the endocrine system (endocrinologist) or a surgeon.
Because appointments can be brief, and because there's often a lot of ground to cover, it's a good idea to be well prepared. Here's some information to help you get ready, and know what to expect from your doctor.
What you can do
- Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
- Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
- Write down key personal information, including any major stresses or recent life changes.
- Make a list of all medications, vitamins or supplements that you're taking.
- Consider taking a family member or friend along. Sometimes it can be difficult to remember all the information provided during an appointment. Someone who accompanies you may remember something that you missed or forgot.
- Write down questions to ask your doctor.
Your time with your doctor is limited, so preparing a list of questions can help you make the most of your time together. List your questions from most important to least important in case time runs out. For carcinoid syndrome, some basic questions to ask your doctor include:
- What is likely causing my symptoms or condition?
- Other than the most likely cause, what are other possible causes for my symptoms or condition?
- What kinds of tests do I need?
- Is my condition likely temporary or chronic?
- What is the best course of action?
- What are the alternatives to the primary approach that you're suggesting?
- I have these other health conditions. How can I best manage them together?
- Are there any restrictions that I need to follow?
- Should I see a specialist? What will that cost, and will my insurance cover it?
- Is there a generic alternative to the medicine you're prescribing me?
- Are there brochures or other printed material that I can take with me? What websites do you recommend?
- What will determine whether I should plan for a follow-up visit?
In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask other questions as they occur to you.
What to expect from your doctor
Your doctor is likely to ask you a number of questions. Being ready to answer them may allow time later to cover other points you want to address. Your doctor may ask:
- When did you first begin experiencing symptoms?
- Have your symptoms been continuous or occasional?
- How severe are your symptoms?
- What, if anything, seems to improve your symptoms?
- What, if anything, appears to worsen your symptoms?
Tests and diagnosis
Your doctor will assess your signs and symptoms in order 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, including the protein chromogranin A, which is released by some carcinoid tumors.
- Imaging tests. Imaging tests also may be used to locate the primary carcinoid tumor and determine whether it has spread. Your doctor may start with a computerized tomography (CT) scan of your abdomen, because most carcinoid tumors are found in the gastrointestinal tract.
Treatments and drugs
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. 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.
- 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. Octreotide may also slow the growth of carcinoid tumors. Side effects of octreotide and lanreotide include diarrhea, abdominal pain and bloating, which may subside over time.
- Biological therapy. An injectable medication called interferon alfa, which stimulates the body's immune system to work better, is sometimes used to slow the growth of carcinoid tumors and to relieve symptoms. Interferon can cause significant side effects, including fatigue and flu-like symptoms.
- 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. Hepatic artery embolization can be risky and the procedure is typically performed only in specialized medical centers. Discuss the benefits and risks with your doctor.
- 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. Radiofrequency ablation and cryotherapy are 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.
Lifestyle and home remedies
Talk to your doctor about self-care measures that may improve your signs and symptoms. Self-care measures can't replace treatment, but they may complement it. Ask your doctor if you should:
- Avoid things that cause skin flushing. Certain substances or situations can trigger flushing, such as alcohol or large meals. Some people experience flushing when they're feeling stressed or upset. Keep track of what causes your flushing, and try to avoid situations that trigger flushing.
- Consider taking a multivitamin. Chronic diarrhea makes it difficult for your body to process the vitamins and nutrients in the food you eat. Ask your doctor whether taking a multivitamin may be a good idea for you.
Coping and support
You may be relieved to finally find an answer to what's been causing your signs and symptoms, but a diagnosis of a rare disease such as carcinoid syndrome can be stressful. As you develop your way of coping with a cancer diagnosis, talk with your health care team about how you feel and consider trying to:
- Find out enough about carcinoid syndrome to make decisions about your care. Ask your doctor questions about your condition. Ask members of your health care team to recommend resources where you can get more information. Knowing about your condition may enable you to better participate in decisions about your care.
- Talk to other people with carcinoid syndrome. Support groups for people with carcinoid syndrome put you in touch with those who have faced the same challenges you are facing. Ask your doctor about groups in your area. Carcinoid syndrome is rare, though, so you may need to connect with people outside your immediate area.
- Take care of yourself. Do what you can to maintain a healthy lifestyle. Eat a diet full of fruits and vegetables. When you feel up to it, work light exercise into your daily routine. Cut extra stress out of your life when possible. Get plenty of sleep so that you feel rested when you wake up. Take care of your body and mind so that you're better able to stick to your cancer treatment plan.
- Abeloff MD, et al. Abeloff's Clinical Oncology. 4th ed. Philadelphia, Pa.: Churchill Livingstone Elsevier; 2008. http://www.mdconsult.com/das/book/body/208746819-4/0/1709/0.html. Accessed May 30, 2012.
- Feldman M, et al. Sleisenger & Fordtran's Gastrointestinal and Liver Disease: Pathophysiology, Diagnosis, Management. 9th ed. Philadelphia, Pa.: Saunders Elsevier; 2010. http://www.mdconsult.com/books/about.do?eid=4-u1.0-B978-1-4160-6189-2..X0001-7--TOP&isbn=978-1-4160-6189-2&about=true&uniqId=229935664-2192. Accessed May 30, 2012.
- Goldman L, et al. Cecil Medicine. 24th ed. Philadelphia, Pa.: Saunders Elsevier; 2012. http://www.mdconsult.com/das/book/body/191371208-2/0/1492/0.html#. Accessed May 30, 2012.
- Goldfinger SE, et al. Treatment of the carcinoid syndrome. http://www.uptodate.com/index. Accessed May 30, 2012.