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Carcinoid Tumor - Overview

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

ON THIS PAGE: You will find some basic information about this disease and the parts of the body it may affect. This is the first page of Cancer.Net’s Guide to Carcinoid Tumors. To see other pages, use the menu on the side of your screen. Think of that menu as a roadmap to this full guide.

About the endocrine system and endocrine tumors

The endocrine system consists of cells that produce hormones. Hormones are chemical substances that are carried through the bloodstream to have a specific regulatory effect on the activity of other organs or cells in the body. Part of the endocrine system is the neuroendocrine system, which is made up of cells that are a cross between traditional hormone-producing cells and nerve cells.

A tumor begins when normal cells change and grow uncontrollably, forming a mass called a tumor. A tumor can be cancerous or benign. A cancerous tumor is malignant, meaning it can spread to other parts of the body. A benign tumor means the tumor will not spread and usually can be removed without it causing much harm.

An endocrine tumor is a mass that affects the parts of the body that produce and release hormones. Because an endocrine tumor develops from cells that produce hormones, the tumor itself can produce hormones and cause serious illness.

About carcinoid tumors

A carcinoid tumor starts in the hormone-producing cells of various organs. Carcinoid tumors most often develop in the gastrointestinal tract, in organs such as the stomach or intestines, or in the lungs. However, a carcinoid tumor can also develop in the pancreas, a man’s testicles, or a woman’s ovaries. More than one carcinoid tumor can occur in the same organ.

Here is a general overview of where carcinoid tumors begin:

  • 39% occur in the small intestine.
  • 15% occur in the rectum.
  • 10% occur in the bronchial system of the lungs.
  • 7% occur in the appendix.
  • 5% to 7% occur in the colon.
  • 2% to 4% occur in the stomach.
  • 2% to 3% occur in the pancreas.
  • About 1% occurs in the liver.
  • They rarely occur in ovaries, testicles, and other organs.

Because carcinoid tumors are a type of neuroendocrine tumor, they can make high levels of neuropeptides and amines (hormone-like substances). However, these substances may not be released in large enough amounts to cause symptoms, or the substances may be defective and not cause symptoms. As a result, a carcinoid tumor can grow slowly for many years without causing symptoms. Although a carcinoid tumor is cancerous, it is often described as "cancer in slow motion."

There are two subtypes of lung carcinoid tumors: typical and atypical. The difference is based on how a tumor processes and makes serotonin (5-HT). Serotonin is a neurotransmitter involved in behavior and depression.

  • A typical lung carcinoid tumor produces high levels of serotonin and chromogranin-A in the blood and high levels of 5-HIAA (a product of serotonin breakdown) in the urine.
  • An atypical lung carcinoid tumor produces normal levels of serotonin and chromogranin-A in the blood and normal levels of 5-HIAA in the urine but high levels of serotonin and 5-HTP (an amino acid) in the urine, and it can make 5-HTP.

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Carcinoid Tumor - Statistics

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

ON THIS PAGE: You will find information about how many people learn they have this type of cancer each year and some general survival information. Remember, survival rates depend on several factors. To see other pages, use the menu on the side of your screen.

It is estimated that 12,000 people are diagnosed with a carcinoid tumor each year in the United States. The number of carcinoid tumors diagnosed has been increasing, but the reason for this is unknown.

About 8,000 of all carcinoid tumors occur in the gastrointestinal tract. Carcinoid tumors make up only 1% of cancers of the gastrointestinal tract, but they make up about 50% of all small intestine cancers. The five-year survival rate is the percentage of people who survive at least five years after the cancer is detected, excluding those who die from other diseases. The five-year survival rate of people with a gastrointestinal carcinoid tumor that has not spread ranges from 65% to 90%, depending on where the carcinoid tumor is located.

Each year, an estimated 4,000 adults in the United States are diagnosed with a lung carcinoid tumor. Carcinoid tumors make up about 4% of cancers of the lung. The overall five-year survival rate of people with a typical lung carcinoid tumor is 85% to 90%. People diagnosed with an atypical lung carcinoid tumor have a five-year survival rate of 50% to 70%.

Cancer survival statistics should be interpreted with caution. These estimates are based on data from thousands of people with this type of cancer in the United States each year, but the actual risk for a particular individual may differ. It is not possible to tell a person how long he or she will live with a carcinoid tumor. Because survival statistics are measured in five-year intervals, they may not represent advances made in the treatment or diagnosis of this cancer. Learn more about understanding statistics.

Source: American Cancer Society.

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Carcinoid Tumor - Medical Illustrations

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

ON THIS PAGE: You will find a basic drawing of the common body parts affected by this disease. To see other pages, use the menu on the side of your screen.

Bladder Cancer Illustration

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Carcinoid Tumor - Risk Factors

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

ON THIS PAGE: You will find out more about the factors that increase the chance of developing this type of tumor. To see other pages, use the menu on the side of your screen.

A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors often influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do. However, knowing your risk factors and talking about them with your doctor may help you make more informed lifestyle and health care choices.

The cause of carcinoid tumors is unknown, and no avoidable risk factors have been found. Lung carcinoid tumors are not caused by or related to smoking.

The following factors may raise a person’s risk of developing a carcinoid tumor:

Family history of multiple endocrine neoplasia type 1. Multiple endocrine neoplasia type 1 (MEN1) is a hereditary condition that increases the risk of developing tumors in the pituitary gland, parathyroid gland, and pancreas. It is estimated that approximately 10% of gastrointestinal carcinoid tumors are associated with MEN1.

Race and gender. Gastrointestinal carcinoid tumors are more common among black people than white people, and black men have a higher risk than black women. Among white people, men and women have the same risk. Race and gender are not a significant risk factor in lung carcinoid tumors, but women tend to live longer after treatment.

Age. For gastrointestinal carcinoid tumors, the average age at diagnosis is 55 to 65. For carcinoid tumors of the appendix, the average age at diagnosis is about 40. For lung carcinoid tumors, the average age at diagnosis is between 45 and 55. Children rarely develop carcinoid tumors.

Other stomach conditions. People with diseases that damage the stomach and reduce acid production have a greater risk of developing a stomach carcinoid tumor. In particular, people with pernicious anemia, a type of anemia in which a person has very large, malformed red blood cells, have a higher risk of developing a stomach carcinoid tumor.

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Carcinoid Tumor - Symptoms and Signs

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

ON THIS PAGE: You will find out more about body changes and other things that can signal a problem that may need medical care. To see other pages, use the menu on the side of your screen.

A carcinoid tumor often causes no symptoms in its early stages and is often found unexpectedly by a surgeon during an unrelated surgery or on x-rays for another condition. Below you will find possible signs and symptoms of gastrointestinal carcinoid tumor, lung carcinoid tumor, carcinoid syndrome, and carcinoid crisis. However, these symptoms may also be caused by a medical condition that is not a carcinoid tumor.

If you are concerned about one or more of the symptoms or signs on any of these lists, please talk with your doctor. Your doctor will ask how long and how often you’ve been experiencing the symptom(s), in addition to other questions. This is to help find out the cause of the problem, called a diagnosis.

Symptoms: gastrointestinal carcinoid tumor

Often, symptoms of a gastrointestinal carcinoid tumor only appear if the tumor spreads to the liver. In that case, the hormone-like substances produced by the tumor can travel throughout the body and cause carcinoid syndrome (see below), a group of symptoms caused by hormones being released by the tumor into the bloodstream. Intestinal carcinoid tumors are most likely to cause symptoms.

People with a gastrointestinal carcinoid tumor may experience the following symptoms or signs:

  • Facial flushing (redness and warm feeling over the face)
  • Diarrhea
  • Abdominal pain caused by blockage of the intestines
  • Asthma
  • Rash
  • Heart disease
  • Intestinal bleeding
  • Pellagra (scale-like skin sores, diarrhea, and mental disturbances)
  • Melena (dark, tarry stools that contain blood and usually indicate there is bleeding somewhere in the gastrointestinal tract)

Symptoms: lung carcinoid tumor

A carcinoid tumor in the lungs causes symptoms that result from hormones bypassing the liver and entering the bloodstream. A lung carcinoid tumor is far less likely to cause carcinoid syndrome (see below) than a gastrointestinal carcinoid tumor.

People with a lung carcinoid tumor may experience the following symptoms or signs:

  • Cough, with or without bloody sputum or phlegm
  • Wheezing
  • Post-obstructive pneumonia (when a tumor blocking a large air passage causes an infection)

Carcinoid syndrome

Carcinoid syndrome is more common in people who have had a carcinoid tumor for many years. Approximately 60% of people with a carcinoid tumor eventually develop carcinoid syndrome.

People with carcinoid syndrome may experience the following symptoms or signs:

  • Facial flushing
  • Sweating
  • Diarrhea
  • Shortness of breath
  • Wheezing or asthma-like symptoms
  • Fast heartbeat
  • Heart murmur
  • Unexplained weight gain
  • Weakness
  • Secondary diabetes
  • Increased body and facial hair
  • High blood pressure and significant fluctuations in blood pressure
  • Neurosis and psychosis—Neurosis is a psychological or behavioral disorder primarily characterized by anxiety. Psychosis is a severe emotional and behavioral disorder that can cause a person’s mental capacity to become very distorted or disorganized, which may interfere with the person’s ability to cope with the demands of everyday life.

Stress, strenuous exercise, and drinking alcohol may make these symptoms worse.

Carcinoid crisis

Carcinoid crisis is a term used when all of the symptoms of carcinoid syndrome occur at the same time. Carcinoid crisis is the most serious and life-threatening complication of carcinoid syndrome. It generally occurs after a person has already experienced some symptoms of carcinoid syndrome. Carcinoid crisis may occur suddenly, or it can be brought on by stress, chemotherapy, or anesthesia. A carcinoid crisis may be prevented and successfully treated with octreotide (Sandostatin), a medication that helps raise low blood pressure and control the production of hormones.

Managing symptoms

If a carcinoid tumor is diagnosed, relieving symptoms remains an important part of cancer care and treatment. This may also be called symptom management, palliative care, or supportive care. Below are some common symptoms of a carcinoid tumor, followed by information on how each can be managed. Be sure to talk with your health care team about symptoms you experience, including any new symptoms or a change in symptoms.

Facial flushing. Avoid stress. Ask your doctor about specific substances and foods, including alcohol, that cause facial flushing so you can avoid them.

Wheezing. Ask your doctor about the use of a bronchodilator, a medication that relaxes the muscles in the lungs to make breathing easier.

Diarrhea. Ask your doctor about the use of anti-diarrheal medications.

Carcinoid syndrome. Ask your doctor about the use of octreotide, which is the most effective single medication against carcinoid syndrome.

Heart problems. Tell your doctor immediately if you think you may have a problem with your heart, and ask about the use of diuretics. Diuretics are drugs that increase the amount of urine by increasing function of the heart.

Learn more about managing common cancer symptoms and treatment side effects.

The next section helps explain what tests and scans may be needed to learn more about the cause of the symptoms. Use the menu on the side of your screen to select Diagnosis, or you can select another section, to continue reading this guide.  

Carcinoid Tumor - Diagnosis

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

ON THIS PAGE: You will find a list of the common tests, procedures, and scans that doctors can use to find out what’s wrong and identify the cause of the problem. To see other pages, use the menu on the side of your screen.

Doctors use many tests to diagnose a tumor and find out if it has spread to another part of the body, called metastasis. Some tests may also determine which treatments may be the most effective. For most types of tumors, a biopsy is the only way to make a definitive diagnosis of cancer. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether the cancer has spread. This list describes options for diagnosing a carcinoid tumor, and not all tests listed will be used for every person. Your doctor may consider these factors when choosing a diagnostic test:

  • Age and medical condition
  • Type of tumor suspected
  • Signs and symptoms
  • Previous test results

Most carcinoid tumors are found unexpectedly when people have x-rays or medical procedures done for reasons unrelated to the tumor. For example, many carcinoid tumors of the appendix are found during an appendectomy (surgery to remove the appendix), while duodenal (the top of the small intestine) and stomach carcinoid tumors are usually found during an endoscopy (see below).

If a doctor suspects a carcinoid tumor, he or she will ask for a complete medical and family history and perform a thorough physical examination. In addition, the following tests may be used to diagnose a carcinoid tumor:

Blood/urine tests. The doctor may need samples of the patient’s blood and urine to check for abnormal levels of hormones and other substances. Urine tests check the amount of 5-HIAA (see the Overview). Measurements of serotonin levels may also be taken. A doctor may be able to diagnose a carcinoid tumor from a urine test alone. A blood test to measure chromogranin-A may be needed since the serum serotonin level often changes and may not be as useful as a chromogranin-A test.

Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. The sample removed during the biopsy is examined by a pathologist. A pathologist is a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease.

Molecular testing of the tumor. Your doctor may recommend running laboratory tests on a tumor sample to identify specific genes, proteins, and other factors unique to the tumor. Results of these tests will help decide whether your treatment options include a type of treatment called targeted therapy (see the Treatment Options section).

Endoscopy. This test allows the doctor to see the lining of the upper digestive system with a thin, lighted, flexible tube called an endoscope. The person may be sedated as the tube is inserted through the mouth, down the esophagus, and into the stomach and small bowel. Sedation is giving medication to become more relaxed, calm, or sleepy. If an abnormality is found, a biopsy will be performed.

A colonoscope is a type of endoscope that is inserted through the anus and into the colon. It can be used to diagnose a tumor in the lower section of the digestive system.

Endoscopic ultrasound. An ultrasound uses sound waves to create a picture of the internal organs. This procedure is often done at the same time as an upper endoscopy. During an endoscopic ultrasound, a machine that produces the sound waves, called a transducer, is inserted into the upper digestive tract through the mouth. The endoscopic ultrasound can show enlarged lymph nodes, which may help indicate a tumor or the stage of the disease.

Bone scan. A bone scan uses a radioactive tracer to look at the inside the bones. The tracer is injected into a patient’s vein. It collects in areas of the bone and is detected by a special camera. Healthy bone appears gray to the camera, and areas of injury, such as those caused by cancer, appear dark.

X-ray. An x-ray is a way to create a picture of the structures inside the body. For instance, a chest x-ray may be taken to look for a carcinoid tumor in the lungs. Sometimes, a carcinoid tumor may not show up on a chest x-ray because of its size or location so the doctor may also recommend other types of scans.

Barium x-rays. During a barium swallow, a person swallows a liquid containing barium, which coats the lining of the esophagus, stomach, and intestines. Then a series of x-rays are taken. The barium makes abnormalities easier to see on the x-ray. If there is an abnormality, an endoscopic biopsy can help make the diagnosis of cancer.

A barium enema may be given before x-rays are taken to show the inner surface of the large intestine. During this test, a barium solution is given through the anus and flows throughout the colon, and then the x-rays are taken.

Computed tomography (CT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. A CT scan can also be used to measure the tumor’s size. Additionally, a CT scan is used to see if the tumor has spread to the liver and to detect a carcinoid tumor in the retroperitoneal (the area behind the abdomen) lymph nodes. Sometimes, a special dye called a contrast medium is given before the scan to provide better detail on the image. This dye can be injected into a patient’s vein or given as a pill to swallow.

Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. MRI can also be used to measure the tumor’s size. A special dye called a contrast medium is given before the scan to create a clearer picture. This dye can be injected into a patient’s vein or given as a pill to swallow.

Radionuclide scanning (OctreoScan). A small amount of a radioactive hormone-like substance that is attracted to carcinoid tumors is injected into a patient’s vein. A special camera is then used to show where the radioactivity has built up in the body. This procedure is useful for identifying where a carcinoid tumor has spread, especially if it has spread to the liver.

Positron emission tomography (PET) scan. A PET scan is a way to create pictures of organs and tissues inside the body. A small amount of a radioactive sugar substance is injected into the patient’s body. This sugar substance is taken up by cells that use the most energy. Because cancer tends to use energy actively, it absorbs more of the radioactive substance. A scanner then detects this substance to produce images of the inside of the body.  However, because a carcinoid tumor grows very slowly, a PET scan may not be as helpful as other tests for diagnosis.

After diagnostic tests are done, your doctor will review all of the results with you. If the diagnosis is cancer, these results also help the doctor describe the tumor; this is called staging.

The next section helps explain the different stages for this type of cancer. Use the menu on the side of your screen to select Stages, or you can select another section, to continue reading this guide.  

Carcinoid Tumor - Stages

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

ON THIS PAGE: You will learn about how doctors describe a tumor’s growth or spread. This is called the stage. To see other pages, use the menu on the side of your screen.

Staging is a way of describing where the tumor is located, if or where it has spread, and whether it is affecting other parts of the body. Doctors use diagnostic tests to find out the cancer’s stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient’s prognosis, which is the chance of recovery. There are different stage descriptions for different types of carcinoid tumors.

Gastrointestinal carcinoid tumor

There is no standard system for staging a gastrointestinal carcinoid tumor, except for a carcinoid tumor in the pancreas, which uses the same staging system as pancreatic cancer. Some doctors use the same system that is used for other cancers of the same organ. For example, a stomach carcinoid tumor may be staged similarly to stomach cancer.

Because there is no standard system, many doctors classify each gastrointestinal carcinoid tumor into one of three general stages:

Localized. The carcinoid tumor has not spread outside of the wall of the primary organ, such as the stomach, colon, or intestine.

Regional spread. The carcinoid tumor has spread through the wall of the primary organ to nearby tissues, such as fat, muscle, or lymph nodes.

Distant spread. The carcinoid tumor has spread to tissues or organs far away from the primary organ, such as the liver, bones, or lungs.

Recurrent. A recurrent tumor is a tumor that has come back after treatment. If there is a recurrence, the cancer may need to be staged again (called re-staging).

Lung carcinoid tumor

The staging of a lung carcinoid tumor is the same as the staging of non-small cell lung cancer (NSCLC). Learn more about lung cancer staging.

Information about the cancer’s stage will help the doctor recommend a treatment plan.  The next section helps explain the treatment options for this type of cancer. Use the menu on the side of your screen to select Treatment Options, or you can select another section, to continue reading this guide.  

Carcinoid Tumor - Treatment Options

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

ON THIS PAGE: You will learn about the different ways doctors treat people with a carcinoid tumor. To see other pages, use the menu on the side of your screen.

This section outlines treatments that are the standard of care (the best proven treatments available) for this specific type of tumor. When making treatment plan decisions, patients are also encouraged to consider clinical trials as an option. A clinical trial is a research study to test a new approach to treatment to evaluate whether it is safe, effective, and possibly better than the standard treatment. Clinical trials may test such approaches as a new drug, a new combination of standard treatments, or new doses of current therapies. Your doctor can help you review all of your treatment options. For more information, see the Clinical Trials and Latest Research sections.

Treatment overview

In cancer care, different types of doctors often work together to create a patient’s overall treatment plan that combines different types of treatments. This is called a multidisciplinary team.

Descriptions of the most common treatment options for a carcinoid tumor are listed below, followed by an outline of treatment options by stage for a gastrointestinal carcinoid tumor. Treatment options and recommendations depend on several factors, including the type and stage of cancer, possible side effects, and the patient’s preferences and overall health. Your care plan may also include treatment for symptoms and side effects, an important part of cancer care. Take time to learn about all of your treatment options and be sure to ask questions about things that are unclear. Also, talk about the goals of each treatment with your doctor and what you can expect while receiving the treatment. Learn more about making treatment decisions.

Surgery

Surgery is the removal of the tumor and surrounding tissue during an operation. A surgical oncologist is a doctor who specializes in cancer surgery. Complete removal of the entire tumor is the standard treatment when possible, and many carcinoid tumors are successfully treated with surgery alone. Often, the surgeon will also remove some healthy tissue around the tumor in hopes of leaving negative margins, which means there are no traces of cancer found in the healthy tissue. When a complete removal of the tumor is not possible, debulking surgery is often used. Debulking surgery removes as much of the tumor as possible and may provide some relief from symptoms.

Talk with your health care team about the possible side effects of the specific operation beforehand and how they will be relieved or managed. Learn more about cancer surgery.

Surgery for a gastrointestinal carcinoid tumor

Local excision. During this operation, the surgeon removes the primary tumor and a margin of healthy tissue around the primary location. Most localized tumors can be surgically removed through a skin incision, but a rectal carcinoid tumor may be removed through the anus. Other gastrointestinal carcinoid tumors can sometimes be removed using an endoscope (see the Diagnosis section).

Electro-fulguration (radiofrequency ablation, RFA). Sometimes used for rectal carcinoid tumors, this treatment destroys the tumor by heating it with an electric current.

Segmental colon resection or hemicolectomy. During this surgery, one-third to one-half of the colon, as well as nearby blood vessels and lymph nodes, are removed.

Low anterior resection. During this surgery, a portion of the upper part of the rectum is removed.

Abdominoperineal resection. This surgery is used for a larger tumor in the lower part of the rectum. It is the removal of the anus, rectum, and part of the colon. After surgery, a colostomy may be used to carry wastes out of the body. A colostomy is an opening from the colon to the outside of the body.

Procedures to treat cancer that has spread to the liver

Liver resection. This surgery removes areas of metastases from the liver. It is not expected to eliminate the cancer, but it often helps relieve or reduce the symptoms of carcinoid syndrome.

Liver transplantation. Liver transplantation is rarely used as a treatment for carcinoid tumor, but it may help younger patients with a carcinoid tumor that began in the liver.

Intratumoral ethanol injection. A CT scan is used to guide a needle into the areas of tumor spread, which are then destroyed by injecting concentrated alcohol through the needle. Liquid nitrogen can also be used to cool the needle and destroy the cells by freezing. These methods are particularly useful if traditional surgical procedures are difficult or impossible to perform.

Hepatic artery occlusion or embolization. These procedures block the tumor’s blood supply by sealing off the blood vessels leading to the tumor. The effectiveness of chemotherapy combined with hepatic artery embolization is not yet clear and continues to be studied. This treatment can cause severe side effects and is only considered when other treatment options are not working.

Surgery for a lung carcinoid tumor

Sleeve resection. The tumor is surgically removed along with parts of the airway above and below the tumor. The airway is then reconnected.

Wedge resection. During this surgery, a small, wedge-shaped piece of the lung is removed. This surgery is used if the tumor is very small.

Lobectomy. During this surgery, an entire lobe of a lung is removed. This surgery is often used if a sleeve resection is not possible because of the size and location of a tumor. A lobectomy is also used if a carcinoid tumor is found at the edge of the lungs, away from large airways.

Pneumonectomy. During this surgery, an entire lung is removed.

Lymph node dissection. Removal of the lymph nodes near the lungs is also common during surgery for a lung carcinoid tumor. Removing the lymph nodes reduces the risk of the carcinoid tumor spreading to other organs.

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to destroy cancer cells. A radiation therapy regimen (schedule) usually consists of a specific number of treatments given over a set period of time. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist.

The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. External-beam radiation therapy is the most common radiation treatment for a carcinoid tumor. It is most often used to relieve symptoms, such as pain, caused by cancer that has spread to the bone as part of palliative care (see below).

A second type of radiation treatment is internal radiation or brachytherapy. Internal radiation therapy is the use of tiny pellets or rods containing radioactive materials that are surgically implanted in or near the site of the tumor. The implant is left in place for several days while the patient stays in the hospital.

Patients receiving radiation therapy may experience fatigue during treatment, and the treated area may become red and dry. Radiation therapy to the chest or neck can cause a dry, sore throat, or a dry cough. Some patients have shortness of breath during radiation therapy. Most side effects go away after the treatment is finished. Learn more about radiation therapy.

Chemotherapy

Chemotherapy is the use of drugs to destroy cancer cells, usually by stopping the cancer cells’ ability to grow and divide. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication.

Systemic chemotherapy is delivered through the bloodstream to reach cancer cells throughout the body. Common ways to give chemotherapy include an intravenous (IV) tube placed into a vein using a needle or in a pill or capsule that is swallowed (orally). A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a set period of time. A patient may receive one drug at a time or combinations of different drugs at the same time.

Chemotherapy for a carcinoid tumor is most often used when the tumor has spread to other organs or is causing severe symptoms. A carcinoid tumor usually does not respond to chemotherapy alone, and other treatments may be necessary.

The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, hair loss, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished.

Learn more about chemotherapy and preparing for treatment. The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions by using the searchable drug databases.

Immunotherapy

Immunotherapy, also called biologic therapy, is designed to boost the body's natural defenses to fight the cancer. It uses materials made either by the body or in a laboratory to improve, target, or restore immune system function. Immunotherapy, such as alpha-interferon and octreotide, may shrink a carcinoid tumor or stop its growth. These therapies work by changing the surface proteins of cancer cells and by slowing their growth. Biologic therapies are also used to treat symptoms by controlling the production of hormones. Learn more about immunotherapy.

Targeted therapy

Targeted therapy is a treatment that targets the cancer’s specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. This type of treatment blocks the growth and spread of cancer cells while limiting damage to healthy cells.

Recent studies show that not all tumors have the same targets. To find the most effective treatment, your doctor may run tests to identify the genes, proteins, and other factors in your tumor. As a result, doctors can better match each patient with the most effective treatment whenever possible. In addition, many research studies are taking place now to find out more about specific molecular targets and new treatments directed at them. Learn more about targeted treatments.

For carcinoid tumors, targeted therapies being researched in clinical trials include drugs that interfere with new blood vessel formation or with specific survival pathways of cancer cells. Research continues on these and other approaches. Talk with your doctor about possible side effects for a specific medication and how they can be managed.

Getting care for symptoms and side effects

Cancer and its treatment often cause side effects. In addition to treatment to slow, stop, or eliminate the cancer, an important part of cancer care is relieving a person’s symptoms and side effects. This approach is called palliative or supportive care, and it includes supporting the patient with his or her physical, emotional, and social needs.

Palliative care can help a person at any stage of illness. People often receive treatment for the cancer and treatment to ease side effects at the same time. In fact, patients who receive both often have less severe symptoms, better quality of life, and report they are more satisfied with treatment.

Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, and other therapies. You may also receive palliative treatments similar to those meant to eliminate the cancer, such as chemotherapy, surgery, and radiation therapy. Talk with your doctor about the goals of each treatment in your treatment plan.

For some people, such as those with lung disease, heart disease, or other specific medical conditions, surgery cannot successfully treat the cancer. In these cases, palliative surgery to relieve symptoms can be helpful, such as removing most of the tumor through a bronchoscope or vaporizing most of it with a laser. Palliative surgery is often used together with radiation therapy (see below). These treatments can help relieve symptoms caused by blocked airways.

Before treatment begins, talk with your health care team about the possible side effects of your specific treatment plan and supportive care options. And during and after treatment, be sure to tell your doctor or another health care team member if you are experiencing a problem so it is addressed as quickly as possible. Learn more about palliative care.

Treatment of a gastrointestinal carcinoid tumor by stage

Localized

Stomach. A localized carcinoid tumor of the stomach can often be completely removed through an endoscope. A tumor larger than 2 centimeters (cm) is removed with a margin of surrounding stomach tissue through an incision in the abdomen. For patients whose carcinoid tumor is stimulated by gastrin, a hormone released by cells of the antrum of the stomach (the part next to the small intestine), the removal of the antrum may be recommended.

Small intestine. Surgery to remove the tumor and surrounding tissue is the most common treatment for a carcinoid tumor smaller than 1 cm located in the small intestine. Surgery for a larger tumor involves removing more surrounding tissue, as well as some surrounding blood vessels and lymph nodes.

Large intestine. The most common treatment for a tumor smaller than 2 cm is the surgical removal of the tumor and surrounding tissue, often done through a colonoscope (see the Diagnosis section). If the tumor is larger than 2 cm, surgery most often involves an incision through the skin.

Appendix. An appendectomy (removal of the appendix) is usually the only treatment needed for a carcinoid tumor smaller than 1.5 cm. For a tumor larger than 2 cm, the removal of about one-third of the colon next to the appendix, along with nearby blood vessels and lymph nodes, is often needed.

Rectum. A rectal carcinoid tumor smaller than 1 cm is treated with electro-fulguration, which destroys the tumor by heating it with an electric current. A tumor larger than 2 cm is more likely to grow and spread quickly, so it is removed using the same procedure as is used for rectal cancer in which some of the healthy tissue of the colon or rectum and some of the nearby lymph nodes are removed.

Regional spread

Whenever possible, the primary tumor and areas of spread to nearby tissues and lymph nodes are removed during surgery. If this is not possible, surgery can help relieve symptoms, such as intestinal blockage.

Distant spread

If cancer has spread to another location in the body, it is called metastatic cancer. Patients with this diagnosis are encouraged to talk with doctors who are experienced in treating this stage of cancer, because there can be different opinions about the best treatment plan. Learn more about seeking a second opinion before starting treatment, so you are comfortable with the treatment plan chosen. This discussion may include clinical trials.

Your health care team may recommend a treatment plan that includes a combination of the types of treatment described above. Surgery is used to relieve symptoms rather than eliminate the cancer at this stage. If distant metastases are not causing symptoms, surgery may not be needed. If carcinoid syndrome is causing symptoms, surgery to remove as much cancer as possible is often recommended. Chemotherapy and radiation therapy may also be offered to help relieve symptoms. Participation in clinical trials is encouraged. Supportive care will also be important to help relieve symptoms and side effects.

For most patients, a diagnosis of metastatic cancer is very stressful and, at times, difficult to bear. Patients and their families are encouraged to talk about the way they are feeling with doctors, nurses, social workers, or other members of the health care team. It may also be helpful to talk with other patients, including through a support group.

Remission and the chance of recurrence

A remission is when cancer cannot be detected in the body and there are no symptoms. This may also be called “no evidence of disease” or NED.

A remission can be temporary or permanent. This uncertainty leads to many survivors feeling worried or anxious that the cancer will come back. While many remissions are permanent, it’s important to talk with your doctor about the possibility of the cancer returning. Understanding the risk of recurrence and the treatment options may help you feel more prepared if the cancer does return. Learn more about coping with the fear of recurrence.

If the cancer does return after the original treatment, it is called recurrent cancer. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence).

When this occurs, a cycle of testing will begin again to learn as much as possible about the recurrence, including whether the tumor’s stage has changed. After testing is done, you and your doctor will talk about your treatment options. Often the treatment plan will include the therapies described above, such as surgery, chemotherapy, and radiation therapy, but they may be used in a different combination or given at a different pace. Your doctor may also suggest clinical trials that are studying new ways to treat this type of recurrent cancer.

People with recurrent cancer often experience emotions such as disbelief or fear. Patients are encouraged to talk with their health care team about these feelings and ask about support services to help them cope. Learn more about dealing with cancer recurrence.

If treatment fails

Recovery from a carcinoid tumor is not always possible. If treatment is not successful, the disease may be called advanced or terminal cancer.

This diagnosis is stressful, and this is difficult to discuss for many people. However, it is important to have open and honest conversations with your doctor and health care team to express your feelings, preferences, and concerns. The health care team is there to help, and many team members have special skills, experience, and knowledge to support patients and their families. Making sure a person is physically comfortable and free from pain is extremely important.

Patients who have advanced cancer and who are expected to live less than six months may want to consider a type of palliative care called hospice care. Hospice care is designed to provide the best possible quality of life for people who are near the end of life. You and your family are encouraged to think about where you would be most comfortable: at home, in the hospital, or in a hospice environment. Nursing care and special equipment can make staying at home a workable alternative for many families. Learn more about advanced cancer care planning.

After the death of a loved one, many people need support to help them cope with the loss. Learn more about grief and loss.

The next section helps explain clinical trials, which are research studies. Use the menu on the side of your screen to select About Clinical Trials, or you can select another section, to continue reading this guide.  

Carcinoid Tumor - About Clinical Trials

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

ON THIS PAGE: You will learn more about clinical trials, which are the main way that new medical approaches are tested to see how well they work. To see other pages, use the menu on the side of your screen.

Doctors and scientists are always looking for better ways to treat patients with a carcinoid tumor. To make scientific advances, doctors create research studies involving volunteers, called clinical trials.

Many clinical trials are focused on new treatments, evaluating whether a new treatment is safe, effective, and possibly better than the current (standard) treatment. These types of studies evaluate new drugs, different combinations of existing treatments, new approaches to radiation therapy or surgery, and new methods of treatment. Patients who participate in clinical trials are often among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment.

There are also clinical trials that study new ways to ease symptoms and side effects during treatment and manage the late effects that may occur after treatment. Talk with your doctor about clinical trials regarding side effects. In addition, there are ongoing studies about ways to prevent the disease.

Patients decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other patients volunteer for clinical trials because they know that these studies are the only way to make progress in treating a carcinoid tumor. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with a carcinoid tumor.

Sometimes people have concerns that, by participating in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill.” The use of placebos in cancer clinical trials is rare. When a placebo is used in a study, it is done with the full knowledge of the participants. Find out more about placebos in cancer clinical trials.

To join a clinical trial, patients must participate in a process known as informed consent. During informed consent, the doctor should list all of the patient’s options so that the person understands how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different from the risks of standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment.

For specific topics being studied for a carcinoid tumor, learn more in the Latest Research section.

Patients who participate in a clinical trial may stop participating at any time for any personal or medical reason. This may include that the new treatment is not working or there are serious side effects. Clinical trials are also closely monitored by experts who watch for any problems with each study. It is important that patients participating in a clinical trial talk with their doctor and researchers about who will be providing their treatment and care during the clinical trial, after the clinical trial ends, and/or if the patient chooses to leave the clinical trial before it ends. 

Cancer.Net offers a lot of information about cancer clinical trials in other areas of the website, including a complete section on clinical trials and places to search for clinical trials for a specific type of cancer.

The next section helps explain the areas of research going on today about this type of cancer. Use the menu on the side of your screen to select Latest Research, or you can select another section, to continue reading this guide.  

Carcinoid Tumor - Latest Research

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

ON THIS PAGE: You will read about the scientific research being done now to learn more about this type of tumor and how to treat it. To see other pages, use the menu on the side of your screen.

Doctors are working to learn more about carcinoid tumors, ways to prevent them, how to best treat them, and how to provide the best care to people diagnosed with this disease. The following areas of research may include new options for patients through clinical trials. Always talk with your doctor about the diagnostic and treatment options best for you.

New treatment approaches. Currently, many clinical trials for carcinoid tumors are studying new drugs and drug combinations. This includes the approaches of targeted therapy and immunotherapy as described in the Treatment Options section.

Genetics. As outlined in the Risk Factors section, family history of MEN1 may play a role in an individual’s risk level for developing a carcinoid tumor. Researchers are investigating MEN1 to learn more about this hereditary risk.

Supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current carcinoid tumor treatments in order to improve patients’ comfort and quality of life.

Looking for More About the Latest Research?

If you would like additional information about the latest areas of research regarding carcinoid tumor, explore these related items that take you outside of this guide:

The next section addresses how to cope with the symptoms of the disease or the side effects of its treatment. Use the menu on the side of your screen to select Coping with Side Effects, or you can select another section, to continue reading this guide.  

Carcinoid Tumor - Coping with Side Effects

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

ON THIS PAGE: You will find out more about steps to take to help cope with physical, social, and emotional side effects. This page includes several links outside of this guide to other sections of this website. To see other pages, use the menu on the side of your screen.

Fear of treatment side effects is common after a diagnosis of a carcinoid tumor, but it may be helpful to know that preventing and controlling side effects is a major focus of your health care team. This is called palliative or supportive care, and it is an important part of the overall treatment plan, regardless of the stage of disease.

Common side effects from each treatment option for carcinoid tumor are described in detail within the Treatment Options section. Learn more about the most common side effects of cancer and different treatments, along with ways to prevent or control them. Side effects depend on a variety of factors, including the cancer’s stage, the length and dosage of treatment(s), and your overall health.

Before treatment begins, talk with your doctor about possible side effects of each type of treatment you will be receiving. Ask which side effects are most likely to happen, when they are likely to occur, and what can be done to prevent or relieve them. And, ask about the level of caregiving you may need during treatment and recovery, as family members and friends often play an important role in the care of a person with a carcinoid tumor. Learn more about caregiving.

In addition to physical side effects, there may be emotional and social effects as well. Patients and their families are encouraged to share their feelings with a member of their health care team who can help with coping strategies, including concerns about managing the cost of your medical care

During and after treatment, be sure to tell the health care team about the side effects you experience, even if you feel they are not serious. Sometimes, side effects can last beyond the treatment period, called a long-term side effect. A side effect that occurs months or years after treatment is called a late effect. Treatment of both types of effects is an important part of survivorship care. Learn more by reading the After Treatment section or talking with your doctor.

The next section helps explain medical tests and check-ups needed after finishing cancer treatment. Use the menu on the side of your screen to select After Treatment, or you can select another section, to continue reading this guide.  

Carcinoid Tumor - After Treatment

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

ON THIS PAGE: You will read about your medical care after treatment for a carcinoid tumor is finished and why this follow-up care is important. To see other pages, use the menu on the side of your screen.

After treatment for a carcinoid tumor ends, talk with your doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your recovery for the coming months and years. This is important because a carcinoid tumor can recur even several years after treatment. The most common place for a second carcinoid tumor to develop is in the gastrointestinal tract, such as the stomach or intestines. Tell your doctor about any new symptoms as soon as you notice them, such as fatigue, breathing problems, or pain in any part of the body.

Patients and families should be aware that a carcinoid tumor is slow growing and may be similar to a chronic illness, which means the patient will receive treatment and follow-up care in cycles on an ongoing basis. Some patients also experience late effects after being treated with octreotide, such as thyroid and gallbladder problems.  

ASCO offers cancer treatment summary forms to help keep track of the cancer treatment you received and develop a survivorship care plan once treatment is completed.

People recovering from a carcinoid tumor are encouraged to follow established guidelines for good health, such as maintaining a healthy weight, not smoking, limiting alcohol, eating a balanced diet, and having recommended cancer screening tests. Talk with your doctor to develop a plan that is best for your needs. Moderate physical activity can help you rebuild your strength and energy level. Your doctor can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about the next steps to take in survivorship, including making positive lifestyle changes.

The next section offers a list of questions you may want to ask. Use the menu on the side of your screen to select Questions to Ask the Doctor, or you can select another section, to continue reading this guide.  

Carcinoid Tumor - Questions to Ask the Doctor

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

ON THIS PAGE: You will find some questions to ask your doctor or other members of your health care team to help you better understand your diagnosis, treatment plan, and overall care. To see other pages, use the menu on the side of your screen.

Talking often with the doctor is important to make informed decisions about your health care. These suggested questions are a starting point to help you learn more about your cancer care and treatment. You are also encouraged to ask additional questions that are important to you. You may want to print this list and bring it to your next appointment, or download Cancer.Net’s free mobile app for an e-list and other interactive tools to manage your care.

  • Where is the carcinoid tumor located?
  • What is the stage of my cancer? What does this mean?
  • Can you explain my pathology report (laboratory test results) to me?
  • Do I have carcinoid syndrome? What does this mean?
  • Is my heart affected by the tumor? How will you monitor my heart’s health and performance?
  • What are my treatment options?
  • What clinical trials are open to me? Where are they located, and how do I find out more about them?
  • Which treatment plan do you recommend? Why?
  • What is the goal of each treatment? Is it to eliminate the cancer, help me feel better, or both?
  • Can surgery remove the entire tumor? If not, can surgery be done to debulk the tumor? What is the benefit of this?
  • Who will be part of my health care team, and what does each member do?
  • Who will be coordinating my overall treatment and follow-up care?
  • What are the possible side effects of this treatment, both in the short term and the long term?
  • How will this treatment affect my daily life? Will I be able to work, exercise, and perform my usual activities?
  • Could this treatment affect my sex life? If so, how and for how long?
  • Could this treatment affect my ability to become pregnant or have children? If so, should I talk with a fertility specialist before treatment begins?
  • If I’m worried about managing the costs related to my cancer care, who can help me with these concerns?
  • What are the chances that the tumor will recur?
  • What follow-up tests will I need, and how often will I need them?
  • What support services are available to me? To my family?
  • Whom should I call for questions or problems?

The next section offers some more resources that may be helpful to you. Use the menu on the side of your screen to select Additional Resources, or you can select another section, to continue reading this guide.  

Carcinoid Tumor - Additional Resources

This section has been reviewed and approved by the Cancer.Net Editorial Board, 03/2014

ON THIS PAGE: You will find some helpful links to other areas of Cancer.Net that provide information about cancer care and treatment. This is the final page of Cancer.Net’s Guide to Carcinoid Tumors. To go back and review other pages, use the menu on the side of your screen.

Cancer.Net includes many other sections about the medical and emotional aspects of cancer, both for the person diagnosed and their family members and friends. This website is meant to be a resource for you and your loved ones from the time of diagnosis, through treatment, and beyond. Here are a few sections that may get you started in exploring the rest of Cancer.Net:

This is the end of Cancer.Net’s Guide to Carcinoid Tumors. Use the menu on the side of your screen to select another section, to continue reading this guide.