What is a carcinoid tumor?

Cancer starts when cells change and grow out of control. The changed or abnormal cells often grow to form a lump or mass called a tumor. Cancer cells can also grow into (invade) nearby areas. And they can spread to other parts of the body. This is called metastasis.

Carcinoid tumors are also called neuroendocrine tumors. It's a rare type of cancer. Carcinoid tumors tend to grow slowly. Few people with these tumors have symptoms. So you may have a carcinoid tumor for many years and never know it.

Carcinoid tumors can grow anywhere in your body where there are hormone-producing (neuroendocrine) cells. Hormones are chemical messengers that travel through your blood. Most carcinoid tumors form in the digestive (gastrointestinal or GI) tract. This is likely because there are more neuroendocrine cells there than anywhere else in the body.

Here's where carcinoid tumors are most often found:

• Small intestine

• Appendix

• Rectum

• Colon

• Stomach

• Esophagus

• Pancreas

• Liver

• Lungs

Who is at risk for a carcinoid tumor?

A risk factor is anything that may increase your chance of having a disease. The exact cause of someone’s cancer may not be known. But risk factors can make it more likely for a person to have cancer. Some risk factors may not be in your control. But others may be things you can change. 

The risk factors for carcinoid tumor include:

• Family history. A family history of certain inherited syndromes (those passed in families) can increase risk. Some of the syndromes linked to carcinoid tumors are multiple endocrine neoplasia type 1, von Hippel-Lindau syndrome, and neurofibromatosis type 1.

• Race and gender. Black people tend to develop more GI carcinoid tumors than white people. Lung carcinoid tumors are more common in white people than people of any other ethnicity. Women tend to have a slightly higher risk than men.

• Other health problems. People with diseases that damage the stomach and reduce acid production have a greater risk of developing stomach carcinoid tumors. People with pernicious anemia also have a higher risk of developing stomach carcinoid tumors.

• Age. People of any age can develop carcinoid tumors. But they are rare in children.

Talk with your healthcare provider about your risk factors for carcinoid tumor and what you can do about them.

What are the symptoms of a carcinoid tumor?

In many cases, carcinoid tumors are nonfunctional and don't cause symptoms. (These tumors don't make hormones and other chemicals.) They might be found during a routine exam or when an exam or surgery is being done for another health issue.

In rare cases, a functional carcinoid tumor can cause a group of symptoms called carcinoid syndrome. This tends to happen after the tumor has spread. The symptoms may be triggered by exercise, stress, or certain foods and drinks, like alcohol (especially red wine), walnuts, pecans, bananas, tofu, and aged cheeses. These are common symptoms:

• Your face gets red (flushed)

• Cramps

• Diarrhea

• Nausea or vomiting

• Asthma-like breathing problems

• Shortness of breath

• Fainting

• Fast heart rate (palpitations)

• Blood pressure changes

• Right-sided valvular heart disease in some people 

Symptoms of tumors that don’t cause carcinoid syndrome depend on where the tumor is and may include:

• Belly (abdominal) pain or pressure

• Change in bowel habits

• Bloody bowel movements

• Chest pain

• Cough

• Wheezing

• Blood-tinged mucus (sputum)

• Nausea and vomiting

• No desire to eat

• Feeling tired all the time (fatigue)

• Weight loss

• A lump or tumor you can feel

Many of these may be caused by other health problems. Still, it's important to see a healthcare provider if you have these symptoms. Only a healthcare provider can tell if you have cancer.

How is a carcinoid tumor diagnosed?

It's hard to diagnose a small carcinoid tumor that’s not causing carcinoid syndrome or other symptoms. These tumors are often found when doing surgery or an exam for another condition. For instance, some carcinoid tumors are found when the appendix is removed due to appendicitis.

These tests may be used to help diagnose carcinoid tumors:

• X-rays and scans. Chest X-ray, CT scan, PET scan, and MRI scan are all useful in diagnosis. These imaging tests can show how big the tumor is and if it has spread.

• Biopsy. A surgical procedure may be needed to take out a tiny piece (sample) of tissue from the carcinoid tumor. This process is called a biopsy. The sample is sent to a lab and tested for cancer cells. A biopsy is important for diagnosis. It's the only way to know for sure that a tumor is cancer. Sometimes it's done by using a lighted, flexible scope to look inside your body. This is called endoscopy. If a tumor is found, a sample of tissue for biopsy can be taken out through the scope. It can also be done by putting a needle through your skin and into the tumor. A syringe is then used to pull out a tissue sample.

• Urine test. If you have a carcinoid tumor that's making too much serotonin hormone, it might show up as an acid in your urine called 5-HIAA (5-hydroxyindoleacetic acid).

• Blood test. A blood test might be done to check for increased levels of hormones or other substances, like serotonin, chromogranin A, neuron-specific enolase, cortisol, or substance P. The blood tests done depend on where the tumor is.

After diagnosis of a carcinoid tumor, you’ll likely need more tests. These help your healthcare providers learn more about the cancer. They can help show if the cancer has grown into nearby tissues or spread to other parts of your body. The test results help your healthcare providers decide the best ways to treat the cancer. 

How is a carcinoid tumor treated?

The best treatment for small carcinoid tumors that have not spread is surgery. Surgery can cure these tumors. Once a tumor has spread or is too big to remove, other treatments may still work well. Here are some treatments that may be used:

• Partial surgery. If a tumor is too big to take it all out, surgery may be used to remove part of it so that other treatments may work better.

• Chemotherapy. Medicines that kill or slow the growth of carcinoid tumor cells may be given into your bloodstream or taken by mouth. Many times, several of these medicines are given together. If a carcinoid tumor has spread into your liver, chemotherapy may be injected right into your liver. Or small pellets may be injected into blood vessels in the liver to block blood flow to the tumor cells.

• Targeted therapy. These medicines act on certain genes or proteins that help cancer cells grow while limiting damage to healthy cells. They might be used to treat certain advanced carcinoid tumors.

• Immunotherapy. This medicine treatment helps your immune system better fight cancer.

• Somatostatin analogs. Octreotide and lanreotide are medicines that are given as a shot (injection). They help control the symptoms of a carcinoid tumor. They may also slow tumor growth.

• PRRT (peptide receptor radionuclide therapy). This treatment is with a radioactive medicine that binds to receptors on some kinds of carcinoid tumors. After binding to the cancer cells, the radiation damages them. This treatment might be used if the somatostatin analogs stop working.

Talk with your healthcare providers about your treatment options. Make a list of questions. Think about the benefits and possible side effects of each option. Discuss your concerns with your healthcare provider before making a decision.

You may also want to get a second opinion before starting treatment. It might help you learn about other treatment options and can give you peace of mind about your treatment decisions. 

What can I do to prevent a carcinoid tumor?

Experts don't know how to prevent carcinoid tumors. None of the known risk factors can be controlled or changed.

How can I manage a carcinoid tumor?

You can help manage a carcinoid tumor by learning as much as you can about it and taking an active role in your treatment. Be sure to follow all your healthcare providers' directions, take your medicines, and keep all your appointments. Tell your healthcare team about any side effects or other problems you're having. There are often ways to help. Here are some other tips:

• Try to follow a healthy, high-protein diet.

• Don’t use alcohol or eat foods that trigger carcinoid symptoms.

• Reduce stress as much as you can.

• Ask your healthcare provider about the medicines you take. Some, like decongestants, asthma inhalers, and antidepressants, may trigger or make carcinoid symptoms worse.

• Join a support group to learn more about carcinoid tumors and share your feelings with others.

• Try mind-body exercises, such as yoga or tai chi to help reduce anxiety and stress.

• Try other ways of managing emotional stress. Guided imagery, meditation, music therapy, and journaling might be helpful.

• Before appointments, write down your questions and bring them with you. For support during your appointments, bring a family member or close friend with you.

Key points about carcinoid tumors

• A carcinoid tumor is a rare type of tumor that often grows slowly.

• Carcinoid tumors are cancer. But in many cases, they don't cause symptoms.

• In rare cases, a carcinoid tumor can cause a group of symptoms called carcinoid syndrome. This often happens after the tumor has spread.

• Carcinoid tumors can grow anywhere there are hormone-producing cells in your body. They most commonly start in the digestive tract.

• The best treatment for small carcinoid tumors that have not spread is surgery.

Next steps

Tips to help you get the most from a visit to your healthcare provider:

• Know the reason for your visit and what you want to happen.

• Before your visit, write down questions you want answered.

• Bring someone with you to help you ask questions and remember what your provider tells you.

• At the visit, write down the name of a new diagnosis and any new medicines, treatments, or tests. Also write down any new instructions your provider gives you.

• Know why a new medicine or treatment is prescribed and how it will help you. Also know what the side effects are.

• Ask if your condition can be treated in other ways.

• Know why a test or procedure is recommended and what the results could mean.

• Know what to expect if you do not take the medicine or have the test or procedure.

• If you have a follow-up appointment, write down the date, time, and purpose for that visit.

• Know how you can contact your healthcare provider if you have questions.