Filed under: Cancer & Chemo
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.
Because carcinoid tumors generally grow slowly, you typically wouldn't experience carcinoid syndrome until the tumors are 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 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. They may be triggered by alcohol, stress and heavy exercise. 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 30 seconds to 30 minutes or longer. Flushing may happen for no obvious reason, though sometimes it can be triggered by eating food 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.
* Rapid heartbeat. Periods of fast heart rate could be a sign of carcinoid syndrome.
Keep in mind that many of these signs and symptoms are more likely the result of a condition other than carcinoid syndrome. Experiencing these symptoms doesn't necessarily mean you have a carcinoid tumor.
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 degrades those 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, which are not degraded 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 much 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.
Only people with carcinoid tumors are at risk of carcinoid syndrome. People with carcinoid tumors are more likely to experience carcinoid syndrome if they have a gastrointestinal carcinoid tumor, especially if the tumor has spread (metastasized) to the liver.
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 is the only treatment to correct 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. Triggers include anesthesia, chemotherapy and other cancer treatments, though carcinoid crisis can occur for no apparent reason. Carcinoid crisis can be fatal. Your doctor may give you medications before surgery or chemotherapy 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 for your appointment. Here's some information to help you get ready, and 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 or your use of certain vitamins or medications.
* 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, as well as any vitamins or supplements, that you're taking.
* Take a family member or friend along. Sometimes it can be difficult to absorb 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?
* Are there any brochures or other printed material that I can take with me? What websites do you recommend visiting?
In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask questions during your appointment at any time that you don't understand something.
What to expect from your doctor
Your doctor is likely to ask you a number of questions. Being ready to answer them may allow you to go over points you want to spend more time on. 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.
* Octreotide (Sandostatin). Injections of the medication octreotide may slow the rate of growth of your carcinoid tumor and reduce the signs and symptoms of carcinoid syndrome. Octreotide controls skin flushing and diarrhea in most people with carcinoid syndrome. Side effects of octreotide include abdominal pain and bloating, diarrhea and nausea, though these effects may subside with time. Some people can't tolerate the side effects of octreotide and must stop taking the drug.
* 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. This drug may be prescribed alone or in combination with octreotide. Interferon also causes significant side effects, including fatigue, bone pain, headaches and vomiting.
* 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 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, especially in people with liver disease, and the procedure is typically performed only in specialized medical centers. Discuss the benefits and risks with your doctor.
* Killing cancer cells by heating or freezing. 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. These treatments might be an option if you have a limited number of liver tumors that are small in size. 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.
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.
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. Your doctor might recommend a multivitamin to supplement your diet. Don't take any vitamins or other dietary supplements without first consulting your doctor.
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 all you can about carcinoid syndrome. 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. Choose healthy meals with plenty 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.