Neuroendocrine Tumor

Overview

About the endocrine system and endocrine tumors

The endocrine system consists of cells that make hormones. Hormones are chemical substances that are formed in the body and carried in the bloodstream to have a specific regulatory effect on the activity of other organs or cells in the body.

A tumor begins when normal cells begin to change and grow uncontrollably, forming a mass. A tumor can be benign or malignant. A benign tumor is not cancerous and usually can be removed without it causing much harm. A malignant tumor is cancerous and can be harmful if not found early and treated. It can spread to and damage the body’s healthy tissues and organs.

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

About neuroendocrine tumors

A neuroendocrine tumor begins in the hormone-producing cells of the body’s neuroendocrine system, which is made up of cells that are a cross between traditional endocrine cells (or hormone-producing cells) and nerve cells. Neuroendocrine cells are found throughout the body in organs, such as the lungs and gastrointestinal tract (such as the stomach and intestines), and perform specific functions, such as regulating the air and blood flow through the lungs and controlling the speed at which food is moved through the gastrointestinal tract.

Types of neuroendocrine tumors

There are many types of neuroendocrine tumors. This section will focus on three specific types: pheochromocytoma, Merkel cell cancer, and neuroendocrine carcinoma. Learn more about other types of cancer that begin in hormone-producing cells, including endocrine tumors, carcinoid tumors,thymoma, thyroid Cancer, and islet cell tumors.

Pheochromocytoma. Pheochromocytoma is a cancer of the chromaffin cells (the cells in the body that release the hormone adrenaline during times of stress). Pheochromocytoma most often occurs in the adrenal medulla, the area inside the adrenal glands. Each person has two adrenal glands, one located on top of each kidney. This type of tumor increases the production of the hormones adrenaline and noradrenaline, which increase blood pressure and heart rate. Pheochromocytoma can be life-threatening because the tumor may release large amounts of adrenaline into the bloodstream after injury. Eighty percent (80%) of people with pheochromocytoma have a tumor on only one adrenal gland, 10% have tumors on both glands, and 10% have a tumor outside of the adrenal glands.

Merkel cell cancer. Merkel cell cancer, also called neuroendocrine carcinoma of the skin or trabular cancer, is a highly aggressive, rare cancer. It starts from hormone-producing cells just beneath the skin and in the hair follicles, and it is found in the head and neck region.

Neuroendocrine carcinoma. Approximately 60% of neuroendocrine tumors are not able to be described as a specific type of cancer other than neuroendocrine carcinoma. Neuroendocrine carcinoma can be found in a number of places in the body, including the lungs, brain, and gastrointestinal tract.

Find out more about basic cancer terms used in this section.

Statistics

An estimated 800 people in the United States are diagnosed with pheochromocytoma each year. The overall five-year relative survival rate (percentage of people who survive at least five years after the cancer is detected, excluding those who die from other diseases) of people with pheochromocytoma is 48%.

More than 1,500 people in the United States are diagnosed with Merkel cell cancer each year. The survival rate of patients with Merkel cell cancer is about 63%.

Cancer survival statistics should be interpreted with caution. These estimates are based on data from thousands of cases of this type of cancer, 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 neuroendocrine tumor. Because the 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: National Cancer Institute Surveillance Epidemiology and End Results (SEER) database, and American Joint Committee on Cancer, Seventh Edition, 2010.  

Risk Factors

A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors can 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 communicating them to your doctor may help you make more informed lifestyle and health care choices.

The following factors can raise a person's risk of developing a neuroendocrine tumor:

Age. Pheochromocytoma is most common between the ages of 40 and 60. Merkel cell cancer is most common in people older than 70.

Gender. Men are more likely to develop pheochromocytoma than women. For every two women who develop pheochromocytoma, three men will develop the disease. Men are also more likely to develop Merkel cell cancer than women.

Race/ethnicity. White people are most likely to develop Merkel cell cancer; however, some black people and people of Polynesian descent have developed the disease.

Family history. Ten percent of pheochromocytoma are linked to hereditary causes. Multiple endocrine neoplasia type 1 (MEN1) is a hereditary condition that increases the risk of developing a tumor of three glands: pituitary, parathyroid, and pancreas. Multiple endocrine neoplasia type 2 (MEN2) is a hereditary condition associated with medullary thyroid cancer and other types of cancer, including pheochromocytoma.

Immune suppression. People with human immunodeficiency virus (HIV) or acquired immune deficiency syndrome (AIDS) and people whose immune systems are lowered because of organ transplantation have a higher risk of developing a neuroendocrine tumor.

Arsenic exposure. Exposure to the poison arsenic may increase the risk of Merkel cell cancer.

Sun exposure. Because Merkel cell cancer often occurs on the sun-exposed areas of the head and neck, many doctors think that sun exposure may be a risk factor for this type of cancer.

Symptoms

People with a neuroendocrine tumor may experience the following symptoms or signs. Sometimes, people with a neuroendocrine tumor do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom or sign on this list, please talk with your doctor.

Pheochromocytoma

• Hypertension (high blood pressure)

• Anxiety attacks

• Fever

• Headaches

• Sweating

• Nausea

• Vomiting

• Clammy skin

• Rapid pulse

• Heart palpitations

Merkel cell cancer

• Painless, firm, shiny lumps on the skin that can be red, pink, or blue in color

Neuroendocrine carcinoma

• Hyperglycemia (too much sugar in the blood, which causes frequent urination, increased thirst, and increased hunger)

• Hypoglycemia (too little sugar in the blood, which causes fatigue, nervousness and shakiness, dizziness or light-headedness, seizures, and fainting)

• Diarrhea

• Persistent pain in a specific area

• Loss of appetite/weight loss

• Persistent cough or hoarseness

• Thickening or lump in any part of the body

• Changes in bowel or bladder habits

• Unexplained weight gain or loss

• Jaundice (yellowing of the skin and whites of the eyes)

• Unusual bleeding or discharge

• Persistent fever or night sweats

• Headache

• Anxiety

• Gastric ulcer disease

• Skin rash

• Nutritional deficiencies

Diagnosis

Doctors use many tests to diagnose cancer and find out if it has metastasized (spread). 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 metastasized. Your doctor may consider these factors when choosing a diagnostic test:

• Age and medical condition

• The type of cancer suspected

• Severity of symptoms

• Previous test results

In addition to a physical examination, the following tests may be used to diagnose a neuroendocrine tumor:

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 from the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease).

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 for increased levels of adrenaline in the body. Large amounts of adrenaline can be a sign of pheochromocytoma. The glucagon stimulation test and clonidine suppression test are blood tests that measure adrenaline levels over a period of time for people who sometimes have symptoms of pheochromocytoma. During the glucagon stimulation test, glucagon (a hormone produced by the pancreas that helps the body process carbohydrates) is injected into a vein, and blood samples are drawn at specific intervals to measure the adrenaline levels. During the clonidine (Catapres) suppression test, a tablet of the drug clonidine is swallowed, and blood samples are taken at regular intervals over the next three hours. Clonidine is used to lower adrenaline levels in the blood, so if these levels do not decrease during testing, it may be a sign of a tumor. Blood pressure and heart rate are carefully monitored during these tests.

X-ray. An x-ray is a picture of the inside of the body. For instance, a chest x-ray can help doctors find out if the cancer has spread to the lungs.

Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then puts these images into a detailed, cross-sectional view that shows any abnormalities or a tumor. Sometimes, a contrast medium (a special dye) is injected into a vein to provide better detail.

Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium may be injected into a patient’s vein to create a clearer picture.

Learn more about what to expect when having common tests, procedures, and scans.

Find out more about common terms used during a diagnosis of cancer.

Staging

Staging is a way of describing a cancer, such as where it is located, if or where it has spread, and if it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the cancer's stage, so staging may not be complete until all 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 (chance of recovery). There are different stage descriptions for different types of cancers.

There is no standard staging system for a neuroendocrine tumor. Staging for pancreatic neuroendocrine tumors is the same as staging for pancreatic cancer.

The following staging system is commonly used for Merkel cell cancer:

Stage IA: The primary tumor is smaller than 2 centimeters (cm) and has not spread to the lymph nodes or other parts of the body.

Stage IB: The primary tumor is larger than 2 cm and has not spread to the lymph nodes or other parts of the body.

Stage II: The cancer has spread to the regional lymph nodes, but has not spread to other parts of the body.

Stage III: The cancer has spread to other parts of the body.

Recurrent: Recurrent cancer is cancer that comes back after treatment.

Source: National Cancer Institute

Treatment

The treatment of a neuroendocrine tumor depends on the size and location of the cancer, whether the cancer has spread, and the person's overall health. In many cases, a team of doctors will work with the patient to determine the best treatment plan.

This section outlines treatments that are the standard of care (the best treatments available) for this specific type of cancer. Patients are also encouraged to consider clinical trials as a treatment option when making treatment plan decisions. A clinical trial is a research study to test a new treatment to prove it is safe, effective, and possibly better than standard treatment. Your doctor can help you review all treatment options. For more information, see the Clinical Trials section.

Descriptions of the most common treatment options for a neuroendocrine tumor are listed below.

Surgery

Surgery is the main treatment for both pheochromocytoma and Merkel cell cancer. A surgical oncologist is a doctor who specializes in treating cancer using surgery. During surgery, the tumor is removed along with a small amount of healthy tissue around the tumor, called the margin. In pheochromocytoma, laparoscopic surgery may be performed. Laparoscopic surgery is a minimally invasive type of surgery that uses three or four small incisions where a laparoscope (a thin, lighted tube) attached to a video camera is inserted through one opening to guide the surgeon. Surgical instruments are inserted through the other openings to perform the surgery. Learn more about cancer surgery.

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to kill cancer cells. 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. When radiation treatment is given using implants, it is called internal radiation therapy, or brachytherapy. A radiation therapy regimen (schedule) usually consists of a specific number of treatments given over a specific time.

Radiation therapy is generally used when a neuroendocrine tumor has spread or is in a location that makes surgery difficult or impossible.

Side effects from radiation therapy may include fatigue, mild skin reactions, upset stomach, and loose bowel movements. Most side effects go away soon after treatment is finished. Learn more about radiation therapy.

Chemotherapy

Chemotherapy is the use of drugs to kill cancer cells. Systemic chemotherapy is delivered through the bloodstream, targeting cancer cells throughout the body. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication. Some people may receive chemotherapy in their doctor's office or outpatient clinic; others may go to the hospital. A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a specific time. There are many clinical trials underway to study new drugs for neuroendocrine tumors. Learn more in the Current Research section.

The side effects of chemotherapy depend on the individual and the dose used, but can include fatigue, risk of infection, nausea and vomiting, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished. Recent studies have found that a combination of fluorouracil (Adrucil, 5-FU), doxorubicin (Adriamycin), and streptozocin (Zanosar) can reduce the side effects of cancer and cancer treatment for some patients. Learn more about chemotherapy and preparing for treatment.

Other medications

Often, pheochromocytoma is treated with alpha-adrenergic blockers (medicines most commonly used to lower blood pressure) in addition to surgery. Beta-blockers may be used to control a fast or irregular pulse.

Targeted therapy is a treatment that targets specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. Some types of targeted therapy may be used to treat a neuroendocrine tumor. Talk with your doctor about targeted therapies and if they are a treatment option for you.

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 searchable drug databases.

Find out more about common terms used during cancer treatment.

Clinical Trials Resources

Doctors and scientists are always looking for better ways to treat patients with neuroendocrine tumors. A clinical trial is a way to test a new treatment to prove that it is safe, effective, and possibly better than a standard treatment. The clinical trial may be evaluating a new drug, a new combination of existing treatments, a new approach to radiation therapy or surgery, or a new method of treatment or prevention. Patients who participate in clinical trials are 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.

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 finding new drugs and other therapies is the only way to make progress in treating neuroendocrine tumors. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with neuroendocrine tumors.

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 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. Learn more about clinical trials , including patient safety, phases of a clinical trial, deciding to participate in a clinical trial, questions to ask the research team, and links to find cancer clinical trials.

For specific topics being studied for neuroendocrine tumors, learn more in the Current Research section.

Side Effects

Cancer and its treatment can cause a variety of side effects. However, doctors have made major strides in recent years in reducing pain, nausea and vomiting, and other physical side effects of cancer treatments. Many treatments used today are less intensive but as effective as treatments used in the past. Doctors also have many ways to provide relief to patients when such side effects do occur.

Fear of treatment side effects is common after a diagnosis of cancer, but it may be helpful to know that preventing and controlling side effects is a major focus of your health care team. Before treatment begins, talk with your doctor about possible side effects of the specific treatments you will be receiving. The specific side effects that can occur depend on a variety of factors, including the type of cancer, its location, the individual treatment plan (including the length and dosage of treatment), and the person’s overall health.

Ask your doctor which side effects are most likely to happen (and which are not), when side effects are likely to occur, and how they will be addressed by the health care team if they do happen. Also, be sure to communicate with the doctor about side effects you experience during and after treatment. Learn more about the most common side effects of cancer and different treatments, along with ways to prevent or control them.

In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. Learn more about the importance of addressing such needs, including concerns about managing the cost of your cancer care.

Learn more about late effects or long-term side effects by reading the After Treatment section or talking with your doctor.

After Treatment

After treatment for a neuroendocrine 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.

While there are no standard guidelines for follow-up care after treatment of a neuroendocrine tumor, people who have had surgery should be seen by their doctor three months after their operation for a physical examination, blood tests, and a CT scan. After that, follow-up care should include a physical examination and blood tests approximately every six to 12 months, with additional imaging studies (such as x-rays) as needed.

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 neuroendocrine tumor are encouraged to follow established guidelines for good health, such as maintaining a healthy weight, not smoking, 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 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 healthy living after cancer.

Find out more about common terms used after cancer treatment is complete.

Current Research

Research for neuroendocrine tumors is ongoing. The following advances may still be under investigation in clinical trials and may not be approved or available at this time. Always discuss all diagnostic and treatment options with your doctor.

New chemotherapy and drug combinations. A study has shown that the drug octreotide (Sandostatin) can slow tumor growth in patients with a neuroendocrine tumor of the midgut (the lower part of the small intestine) that has spread. Drugs used in chemotherapy, such as fluorouracil (Adrucil, 5-FU), leucovorin (Wellcovorin), and oxaliplatin (Eloxatin), work in different ways to stop the growth of tumor cells, either by killing the cells or by stopping them from dividing.

Targeted therapy and combined treatments. Monoclonal antibodies are a type of targeted therapy that can block tumor growth in different ways. Some block the ability of tumor cells to grow and spread. Others find tumor cells and help kill them or carry tumor-killing substances to them.

Bevacizumab (Avastin) is a monoclonal antibody called an antiangiogenic that helps stop blood vessels from forming and feeding the tumor. When combined with chemotherapy, it may kill more tumor cells than chemotherapy alone. Bevacizumab may also stop the growth of neuroendocrine tumors by blocking blood flow to the tumor. Another antiangiogenic being studied for neuroendocrine tumors is sorafenib (Nexavar), which may stop the growth of tumor cells by blocking some of the enzymes needed for cell growth and by blocking blood flow to the tumor.

Another combination of a targeted therapy and chemotherapy being studied is everolimus (Afinitor) with octreotide to help slow the growth of advanced low-grade neuroendocrine tumors that cannot be removed surgically.

New types of treatment. Antineoplastons are made out of mostly peptides and amino acids from blood and urine that may stop or slow the growth of cancer cells, while leaving other cells unaffected.

To find clinical trials specific to your diagnosis, talk with your doctor or search online clinical trial databases now.

Questions to Ask the Doctor

Regular communication with your doctor is important in making informed decisions about your health care. Consider asking the following questions of your doctor:

• What type of neuroendocrine tumor do I have?

• What is the stage of my cancer? What does this mean?

• Can you explain my pathology report (laboratory test results) to me?

• How much experience do you have in treating this type of cancer?

• What organs does the cancer affect?

• What are my treatment options?

• What clinical trials are open to me?

• Can surgery be done to remove all of my cancer?

• What treatment do you recommend? Why?

• What are the possible side effects of this treatment, both in the short term and the long term?

• May I have some reading materials on my cancer and medications? Where can I get more information on my diagnosis and treatment?

• How will this treatment affect my daily life? Will I be able to work, exercise, and perform my usual activities?

• Are the side effects permanent?

• What medications will reduce side effects?

• If I’m worried about managing the costs related to my cancer care, who can help me with these concerns?

• What follow-up tests will I need, and how often will I need them?

• What support services are available to me? To my family?

Patient Information Resources

In addition to Cancer.Net, there are other sources of information about this type of cancer available online. Cancer.Net maintains a list of national, not-for-profit organizations that may be helpful in finding additional information, services, and support. As always, be sure to talk with your doctor about questions you may have about information you find about this disease.

View organizations that offer information on this specific type of cancer.

Carcinoid Tumor

Overview

About the endocrine system and endocrine tumors


The endocrine system consists of cells that produce hormones. Hormones are chemical substances that are formed in the body and carried in the bloodstream to have a specific regulatory effect on the activity of other organs or cells in the body.


A tumor begins when normal cells begin to change and grow uncontrollably, forming a mass. A tumor can be benign or malignant. A benign tumor is not cancerous and usually can be removed without it causing much harm. A malignant tumor is cancerous and can be harmful if not found early and treated. It can spread to and damage the body’s healthy tissues and organs.

An endocrine tumor is a growth that affects the parts of the body that secrete hormones. Because an endocrine tumor arises 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, mostly the gastrointestinal tract (such as the stomach and intestines) and lungs, but also the pancreas, testicles (in males) or ovaries (in females). More than one carcinoid tumor can occur within the same organ. The cause of a carcinoid tumor is unknown.

A carcinoid tumor is a type of neuroendocrine tumor, which means it starts in cells of the neuroendocrine system that make hormones. A carcinoid tumor can make high levels of neuropeptides and amines (hormone-like substances); however, they may not be released in large enough amounts to cause symptoms or they may be defective and not cause symptoms. A carcinoid tumor can grow slowly for many years without causing symptoms. Although a carcinoid tumor is cancerous, it is often referred to as "cancer in slow motion."

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% occur in the liver

• Rarely in ovaries, testicles, and other organs

Carcinoid tumors make up only 1% of cancers of the gastrointestinal tract, but make up about 50% of all small intestine cancers.

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, a neurotransmitter involved in behavior and depression):

• A typical lung carcinoid tumor has high levels 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 has 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 can make 5-HTP.

Find out more about basic cancer terms used in this section.

Statistics

It is estimated that 11,000 to 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 two-thirds of all carcinoid tumors occur in the gastrointestinal tract. If the tumor can be surgically removed and has not spread outside of the organ where it started, the overall five-year relative survival rate (the percentage of people who survive after the cancer is detected, excluding those who die from other diseases) for people with gastrointestinal carcinoid tumors ranges from 70% to 90%. The survival is lower if cancer has spread.

Each year, an estimated 3,000 adults in the United States are diagnosed with lung carcinoid tumors. The five-year relative survival rate of people with typical lung carcinoid tumors is 85% to 90%. Those diagnosed with atypical lung carcinoid tumors have a five-year relative survival rate of 50% to 60%.

Cancer survival statistics should be interpreted with caution. These estimates are based on data from thousands of cases of this type of cancer in the United States and may not apply to a single person. It is not possible to tell a person how long he or she will live with carcinoid tumors. 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 and the National Cancer Institute.

Risk Factors

A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors can 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 communicating them to 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 three different glands: pituitary, parathyroid, 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 a stomach carcinoid tumor.

Symptoms

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.

Symptoms: gastrointestinal carcinoid tumor

Often, symptoms of a gastrointestinal carcinoid tumor only appear if the tumor metastasizes (spreads) to the liver. In that case, the hormone-like substances from 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. Sometimes, people with a gastrointestinal carcinoid tumor do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom or sign on this list, please talk with your doctor.

• 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 elsewhere in the gastrointestinal tract)

Symptoms: lung carcinoid tumor

A carcinoid tumor in the lungs causes symptoms caused by 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. Sometimes, people with a lung carcinoid tumor do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom or sign on this list, please talk with your doctor.

• 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 carcinoid tumors eventually develop carcinoid syndrome.

People with carcinoid syndrome may experience the following symptoms or signs. Sometimes, these symptoms may be caused by a medical condition that is not carcinoid syndrome. If you are concerned about a symptom or sign on this list, please talk with your doctor.

• 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

• Neurosis (a psychologic or behavioral disorder primarily characterized by anxiety) and psychosis (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 worsen these symptoms.

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 and is generally found in people who already experienced some symptoms of carcinoid syndrome. Carcinoid crisis may occur suddenly, or it can be brought on with stress, chemotherapy, or anesthesia. A carcinoid crisis may be prevented and successfully treated with octreotide (Sandostatin), a medication that can help to increase low blood pressure and control the production of hormones.

Managing symptoms

Below are some common symptoms of a carcinoid tumor, followed by information on how each can be managed. Talk with your doctor about each symptom and how it can be treated. Learn more about managing common cancer symptoms and treatment side effects.

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

Wheezing. Ask your doctor about the use of a bronchodilator (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 (the most effective single medication against carcinoid syndrome).

Heart problems. Tell your doctor immediately and ask about the use of diuretics (drugs that increase the amount of urine; diuretics act by increasing function of the heart).

Diagnosis

Doctors use many tests to diagnose a tumor and find out if it has metastasized (spread). 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 metastasized. Your doctor may consider these factors when choosing a diagnostic test:

• Age and medical condition

• The type of cancer suspected

• Severity of 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 appendectomies (surgery to remove the appendix); duodenal (the top of the small intestine) and stomach carcinoid tumors are usually found during endoscopies (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 measurement of 5-HIAA. Measurements for 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 from the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease).

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. If an abnormality is found, a biopsy will be performed.

A colonoscope is a type of endoscope that is inserted through the anus into the colon. It can be used to diagnose tumors 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 the upper endoscopy. In an endoscopic ultrasound, a transducer (a machine that produces the sound waves) is inserted into the upper digestive tract through the mouth. The endoscopic ultrasound can show enlarged lymph nodes, which may indicate a tumor or advanced disease.

Bone scan. A bone scan uses a radioactive tracer to look at the inside of 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 picture of the inside of 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 a computed tomography (CT or CAT) scan (see below).

Barium x-rays. In a barium swallow, a person swallows a liquid containing barium and then a series of x-rays are taken. The barium coats the lining of the esophagus, stomach, and intestines, so abnormalities are 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, then the x-rays are taken.

Computed tomography (CT or CAT) 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 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 contrast medium (a special dye) is injected into a patient’s vein to provide better detail.

Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium may be injected into a patient’s vein to create a clearer picture.

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 accumulates. This procedure is useful for 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 substance is injected into a patient’s body. This substance is absorbed mainly by organs and tissues that use the most energy. Because a tumor 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 carcinoid tumors grow very slowly, a PET scan may not be as helpful as other tests for diagnosis.

Learn more about what to expect when having common tests, procedures, and scans.

Find out more about common terms used during a diagnosis of cancer.

Staging

Staging is a way of describing a cancer, such as where it is located, if or where it has spread, and if it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine 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 (chance of recovery). There are different stage descriptions for different types of cancer.

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 treated similar to stomach cancer.

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

Localized spread. 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 and 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.

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.

Recurrent: A recurrent tumor is a tumor that comes back after treatment.

Treatment

The treatment of a carcinoid tumor depends on the size and location of the tumor, whether the tumor has spread, and the patient’s overall health. In many cases, a team of doctors will work with the patient to determine the best treatment plan.

This section outlines treatments that are the standard of care (the best treatments available) for this specific type of cancer. Patients are also encouraged to consider clinical trials as a treatment option when making treatment plan decisions. A clinical trial is a research study to test a new treatment to prove it is safe, effective, and possibly better than standard treatment. Your doctor can help you review all treatment options. For more information, see the Clinical Trials section.

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.

Surgery

Many carcinoid tumors are successfully treated with surgery only. A surgical oncologist is a doctor who specializes in treating cancer using surgery. Complete removal of the entire tumor is the standard treatment when possible. Often, the surgeon will remove some normal tissue around the tumor in hopes of leaving negative margins (no trace of cancer 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. 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 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 Diagnosis).

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 larger tumors in the lower part of the rectum. It is the removal of the anus, rectum, and part of the colon. After surgery, a colostomy (an opening from the colon to the outside of the body) may be used to carry urine and other waste outside of the body.

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

Liver transplantation. Liver transplantation is rarely used in the treatment of carcinoid tumors, but it may help younger patients with carcinoid tumors that begin in the liver.

Procedures to treat liver metastases

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 kill 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 lowers the risk of the carcinoid tumor spreading to other organs.

Palliative surgery

For some people, such as those with lung diseases, heart disease, or other 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 with radiation therapy (see below). These treatments can help relieve symptoms caused by blocked airways. Learn more about palliative care.

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to kill cancer cells. A radiation therapy regimen (schedule) usually consists of a specific number of treatments given over a specific 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 carcinoid tumors, and it is most often used to relieve symptoms (such as pain) from cancer spread to the bone.

When radiation treatment is given using implants, it is called 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 and 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 kill cancer cells. Systemic chemotherapy is delivered through the bloodstream, targeting cancer cells throughout the body. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication. Some people may receive chemotherapy in their doctor's office or outpatient clinic; others may go to the hospital. A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a specific time.

Chemotherapy for a carcinoid tumor is most often used when the tumor has spread to other organs or is causing severe symptoms. This type of tumor rarely responds to chemotherapy alone, and other treatments may be necessary.

The side effects of chemotherapy depend on the individual and the dose used, but can include fatigue, risk of infection, nausea and vomiting, 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 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 either made by the body or in a laboratory to bolster, 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 specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. Targeted therapies under investigation include drugs that interfere with new vessel formation or with specific survival pathways of cancer cells. Learn more about targeted treatments.

Treatment of a gastrointestinal carcinoid tumor by stage (localized, regional spread, or distant spread)

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 carcinoid tumors (smaller than 1 cm) in the small intestine. Surgery for larger tumors involves removing more surrounding tissue, as well as some surrounding blood vessels and lymph nodes.

Large intestine. The most common treatment for tumors smaller than 2 cm is the surgical removal of the tumor and surrounding tissue, often done through a colonoscope (see Diagnosis). 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 carcinoid tumors smaller than 1.5 cm. For tumors 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. Arectal carcinoid tumor smaller than 1 cm is treated with electro-fulguration (a procedure that destroys the tumor by heating it with an electric current). Tumors larger than 2 cm are more likely to grow and spread quickly, so they are removed using the same procedure as rectal cancer, where some of the healthy tissue of the colon or rectum and some of the nearby lymph nodes are removed.

Regional spread

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

Distant spread

Surgery is used to relieve symptoms, as it is not a curative therapy 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 may also be offered to help relieve symptoms.

Find out more about common terms used during cancer treatment.

Clinical Trials Resources

Doctors and scientists are always looking for better ways to treat patients with a carcinoid tumor. A clinical trial is a way to test a new treatment to prove that it is safe, effective, and possibly better than a standard treatment. The clinical trial may be evaluating a new drug, a new combination of existing treatments, a new approach to radiation therapy or surgery, or a new method of treatment or prevention. Patients who participate in clinical trials are 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.

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 finding new drugs and other therapies is 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 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. Learn more about clinical trials, including patient safety, phases of a clinical trial, deciding to participate in a clinical trial, questions to ask the research team, and links to find cancer clinical trials.

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

Side Effects

A tumor and its treatment can cause a variety of side effects. However, doctors have made major strides in recent years in reducing pain, nausea and vomiting, and other physical side effects of tumor treatments. Many treatments used today are less intensive but as effective as treatments used in the past. Doctors also have many ways to provide relief to patients when such side effects do occur.

Fear of treatment side effects is common after a diagnosis of a tumor, but it may be helpful to know that preventing and controlling side effects is a major focus of your health care team. Before treatment begins, talk with your doctor about possible side effects of the specific treatments you will be receiving. The specific side effects that can occur depend on a variety of factors, including the type of tumor, its location, the individual treatment plan (including the length and dosage of treatment), and the person’s overall health.

Ask your doctor which side effects are most likely to happen (and which are not), when side effects are likely to occur, and how they will be addressed by the health care team if they do happen. Also, be sure to communicate with the doctor about side effects you experience during and after treatment. Learn more about the most common side effects of cancer and different treatments, along with ways to prevent or control them.

In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. Learn more about the importance of addressing such needs, including concerns about managing the cost of your cancer care.

Learn more about late effects or long-term side effects by reading the After Treatment section or talking with your doctor.

After Treatment

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 is the gastrointestinal tract (stomach and 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 carcinoid tumors are slow-growing and may be similar to a chronic illness. Some patients also experience late effects (long-term side effects) after being treated with octreotide (Sandostatin), 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, 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 healthy living after cancer.

Find out more about common terms used after cancer treatment is complete.

Current Research

Research for carcinoid tumors is ongoing. Advances may still be under investigation in clinical trials and may not be approved or available at this time. Always discuss all diagnostic and treatment options with your doctor.

New treatment approaches. Currently, many clinical trials for carcinoid tumors are being done with newer drugs and drug combinations. This includes the approaches of targeted therapy and immunotherapy as described under Treatment.

Genetics. As outlined in Risk factors, family history of this disease may play a role in an individual’s risk level. Researchers are investigating MEN1 to learn more about this hereditary risk.

Supportive care. Clinical trials are underway to find better ways of reducing side effects of treatment to improve patients’ quality of life.

To find clinical trials specific to your diagnosis, talk with your doctor or search online clinical trial databases now.

Questions to Ask the Doctor

Regular communication with your doctor is important in making informed decisions about your health care. Consider asking the following questions of your doctor:

• 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 performance?

• What are my treatment options?

• What clinical trials are open to me?

• Can surgery be done to remove all of the tumor? If not, can surgery be done to debulk the tumor? What advantages will this produce?

• Which treatment plan do you recommend? Why?

• 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?

• If I’m worried about managing the costs related to my cancer care, who can help me with these concerns?

• What new research is available for carcinoid tumors?

• 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?

Patient Information Resources

In addition to Cancer.Net, there are other sources of information about this type of cancer available online. Cancer.Net maintains a list of national, not-for-profit organizations that may be helpful in finding additional information, services, and support. As always, be sure to talk with your doctor about questions you may have about information you find about this disease.

View organizations that offer information on this specific type of cancer.

Multiple Endocrine Neoplasia Type 1

Overview

What is multiple endocrine neoplasia type 1?

Multiple endocrine neoplasia type 1 (MEN1) is a hereditary condition associated with tumors of the endocrine (hormone producing) glands. MEN1 was originally known as Wermer syndrome. The most common tumors seen in MEN1 involve the parathyroid gland, islet cells of the pancreas, and pituitary glands. Other endocrine tumors seen in MEN1 include adrenal cortical tumors and carcinoid tumors, as well as tumors in other parts of the digestive tract.

Non-endocrine tumors are also seen in MEN1; these tumors can include facial angiofibroma (tumor of blood vessels and fibrous tissue); collagenoma (flesh-colored tumor on the skin); lipoma (fatty tumor); meningioma (tumor from nervous system tissue); ependymoma (tumor from nervous system tissue); and leiomyoma (smooth muscle tumor).

The majority of tumors in people with MEN1 are benign (noncancerous), however, approximately one-third of islet cell tumors and mediastinal carcinoid tumors are malignant (cancerous, meaning it can spread to other parts of the body). A malignant islet cell tumor spreads to the liver most commonly; mediastinal carcinoid tumors may grow locally or spread to the liver, lung, or other locations.

In addition, there is a broad spectrum of clinical syndromes associated with increased hormone production by these tumors. These include increased production of:

• prolactin (abnormal milk production by the breast and lack of menstruation in women)

• growth hormone (excessive growth of the jaw and other soft tissues)

• adrenocorticotropic hormone (excessive cortisol production) by pituitary tumors

• gastrin (causing stomach ulcers)

• glucagon (causing diabetes mellitus)

• vasoactive intestinal peptide (VIPoma; causing watery diarrhea) by islet cell tumors of the pancreas

• parathyroid hormone (causing high blood calcium and kidney stones) by parathyroid tumors


What causes MEN1?

MEN1 is a genetic condition. This means that the cancer risk and other features of MEN1 can be passed from generation to generation in a family. The gene associated with MEN1 is also called MEN1. A mutation (alteration) in the MEN1 gene gives a person an increased risk of developing endocrine tumors and other symptoms of MEN1. In individuals without an identifiable MEN1 gene mutation, a small percentage have been found to have germline mutations (alterations in the body’s egg or sperm cells that become incorporated into the DNA of every cell in the body of the offspring) of a class of proteins called cyclin-dependent kinase inhibitors (CDKIs) that regulate cell growth and division. Research is ongoing to learn more about MEN1.

How is MEN1 inherited?

Normally, every cell has two copies of each gene: one inherited from the mother and one inherited from the father. MEN1 follows an autosomal dominant inheritance pattern, in which a mutation happens in only one copy of the gene. This means that a parent with a gene mutation may pass along a copy of their normal gene or a copy of the gene with the mutation. Therefore, a child who has a parent with a mutation has a 50% chance of inheriting that mutation. A brother, sister, or parent of a person who has a mutation also has a 50% chance of having the same mutation. A tumor develops in the affected individual when the normal copy of the gene (inherited from the unaffected parent) is lost or mutated (damaged) in a specific cell type during normal cell division.

How common is MEN1?

It is estimated that about one in 30,000 people has MEN1. About 10% of people with MEN1 do not have a family history of the condition; they have a de novo (new) mutation in the MEN1 gene.

How is MEN1 diagnosed?

MEN1 is suspected when a person has at least two of the most common tumors listed.

• Parathyroid

• Pancreatic

• Pituitary


If a person has a family history of MEN1, they are suspected of also having MEN1 if they have a parathyroid, pancreatic, or pituitary tumor. Genetic testing for mutations in the MEN1 gene is available for people suspected to have MEN1. A mutation in the MEN1 gene is found in about 80% to 90% of families diagnosed with MEN1. Approximately 65% of people with two or more tumors associated with MEN1, but no family history, will have a mutation in the MEN1 gene.

What are the estimated cancer risks associated with MEN1?

Approximately one-third of islet cell tumors of the pancreas are cancerous. If the cancer has spread, the most common site of spread is the liver. A small percentage of mediastinal carcinoid tumors are malignant and spread to local (nearby) lymph nodes or to the liver, lung, or other locations.

What are the screening options for MEN1?

Current suggested screening for people who are known or suspected to have MEN1 includes:

Genetic Testing

• Genetic testing is available. It should be considered for children or young adults who are members of a family diagnosed with MEN1 and an identified mutation of the MEN1 gene to determine which children/young adults should have the screening studies described below. In a family with an identified mutation of the MEN1 gene, children with a genetic test showing no mutation (expected to be 50% of children born to an individual affected with MEN1) may not need the screening tests described below. Certified genetic testing laboratories for this condition can be found at www.genetests.org.


Diagnostic Studies

• Regular blood tests (every 1 to 3 years) for serum prolactin, insulin-like growth factor 1 (IGF-1) level, fasting glucose, insulin, and proinsulin, beginning at age 5 to 10 years.

• A yearly ionized calcium level test, beginning at age 8

• Regular blood tests for fasting gastrin level and fasting and meal stimulated pancreatic polypeptide (PP), fasting VIP, and glucagons, beginning at age 20.

• Magnetic resonance imaging (MRI) scan of the brain, every three years, beginning between ages 5 to 10, or at any time the results of the tests for serum prolactin or insulin-growth factor is abnormal.

• MRI or computed tomography (CT) scan of the chest and abdomen, every two to three years, beginning at age 20 or when the serum gastrin, PP, or VIP is noted to be abnormal.


Screening guidelines may change over time as new technologies are developed and more is learned about MEN1. It is important to talk with your doctor about appropriate screening tests.

What are the treatment options for the endocrine tumors?

Most of these tumors are treated with surgery or by taking a medicine that suppresses growth or function of the tumor. The most challenging treatment issues relate to the pancreatic islet cell tumors. In addition to its role in normal digestion, the pancreas regulates the level of blood glucose through insulin production. Removal of the pancreas will cause diabetes mellitus, a condition that can lead to significant health problems. Doctors must balance the benefits of pancreatic removal in a person with MEN1 (such as the prevention of development of cancer spread) against the risks of diabetes mellitus. Carcinoid tumors should be surgically removed early in the development of the tumor. Learn more about treatment for islet cell tumors and carcinoid tumors.

Learn more about what to expect when having common tests, procedures, and scans.

Find out more about common terms used during a diagnosis of cancer.

Questions to ask the doctor

If you are concerned about your risk of cancer, talk with your doctor. Consider asking the following questions of your doctor:

• What is my risk of developing cancer?

• What can I do to reduce my risk of cancer?

• What are my options for cancer screening?


If you are concerned about your family history and think you or other family members may have MEN1, consider asking the following questions:

• Does my family history increase my risk of developing cancer?

• Should I meet with a genetic counselor?

• Should I consider genetic testing?


Additional resources

Guide to Endocrine Tumor


Guide to Carcinoid Tumor


Guide to Islet Cell Tumor


Guide to Parathyroid Tumor


Guide to Pituitary Tumor


What to Expect When You Meet With a Genetic Counselor


National Cancer Institute

www.cancer.gov


American Cancer Society

www.cancer.org


CancerCare

www.cancercare.org


To find a genetic counselor in your area, ask your doctor or visit these websites:

National Society of Genetic Counselors

www.nsgc.org


National Cancer Institute

www.cancer.gov/search/geneticsservices

Multiple Endocrine Neoplasia Type 2

Overview

What is multiple endocrine neoplasia type 2?

Multiple endocrine neoplasia type 2 (MEN2) is a hereditary condition associated primarily with medullary thyroid carcinoma, a specific type of thyroid cancer. MEN2 is classified into three subtypes based on symptoms.

• Familial medullary thyroid carcinoma (FMTC), which affects 5% to 35% of MEN2 families

 - Medullary thyroid carcinoma only

• MEN2A, which affects 60% to 90% of MEN2 families

 - Medullary thyroid carcinoma

 - Pheochromocytoma (a typically [benign] noncancerous tumor of the adrenal glands)

 - Parathyroid adenoma (benign tumor) or hyperplasia (increased size) of the parathyroid gland


• MEN2B, which affects 5% of MEN2 families

 - Medullary thyroid carcinoma

 - Pheochromocytoma

 - Mucosal neuroma (benign tumor of nerve tissue on the tongue and lips)

 - Digestive problems

 - Muscle, joint, and spinal problems

 - Typical facial features, including swollen lips and thick eyelids


What causes MEN2?

MEN2 is a genetic condition. This means that the cancer risk and other features of MEN2 can be passed from generation to generation in a family. The gene associated with MEN2 is called RET. A mutation (alteration) in the RET gene gives a person an increased risk of developing medullary thyroid cancer and other symptoms of MEN2. Research is ongoing to learn more about MEN2.

How is MEN2 inherited?

Normally, every cell has two copies of each gene: one inherited from the mother and one inherited from the father. MEN2 follows an autosomal dominant inheritance pattern, in which a mutation happens in only one copy of the gene. This means that a parent with a gene mutation may pass along a copy of their normal gene or a copy of the gene with the mutation. Therefore, a child who has a parent with a mutation has a 50% chance of inheriting that mutation. A brother, sister, or parent of a person who has a mutation also has a 50% chance of having the same mutation. If a child inherits the mutant RET gene from an affected parent, there is almost a 100% chance of developing medullary thyroid carcinoma and other symptoms of this syndrome during his or her lifetime.

How common is MEN2?

It is estimated that about one in 30,000 people has MEN2. About 50% of people with MEN2B do not have any family history of the condition. They have a de novo (new) mutation in the RET gene. Less than 5% of people with MEN2A are thought to have a de novo mutation in the RET gene.

How is MEN2 diagnosed?

FMTC is suspected in families with two or more cases of medullary thyroid cancer and no evidence of parathyroid or adrenal gland problems.

MEN2A is suspected when there are at least two of the three common tumors (medullary thyroid cancer, pheochromocytoma, or parathyroid adenoma) in one person or close relatives. Close relatives are generally considered to be parents, siblings, and children.

MEN2B is suspected in children with mucosal neuromas and typical facial features of a person with MEN2B. Medullary thyroid cancer can occur very early in childhood.

Genetic testing for mutations in the RET gene is available. It is recommended for people with a family history of FMTC, MEN2A, and MEN2B, and anyone diagnosed with medullary thyroid cancer. Mutations in the RET gene are found in more than 95% of families with MEN2A or MEN2B, and in more than 85% of families with FMTC. Certified genetic testing laboratories for this condition can be found at www.genetests.org.

What are the estimated cancer risks associated with MEN2?

The risk of medullary thyroid cancer in people with MEN2 is nearly 100% for all subtypes.

What are the screening options for MEN2?

Due to the high risk of medullary thyroid cancer, RET gene testing is recommended for children at risk for any of the MEN2 subtypes. If a mutation is found, the thyroid gland should be surgically removed to prevent cancer from developing. This surgery is recommended by age 3 in children with MEN2B and by age 6 in children with MEN2A or FMTC. The procedure should be performed by a surgeon with experience in both thyroid surgery in children and with the management of medullary thyroid carcinoma.

Additional screening recommendations may include:

• Yearly blood tests for ionized calcium and parathyroid hormone levels, beginning in childhood (MEN2A)

• Yearly blood tests for catecholamines and catecholamine metabolites (metanephrine and normetanephrine), beginning in childhood (FMTC, MEN2A, MEN2B)

• Magnetic resonance imaging (MRI) or computerized tomography (CT or CAT) scan of the abdomen to detect pheochromocytomas, as needed


Screening guidelines may change over time as new technologies are developed and more is learned about MEN2. It is important to talk with your doctor about appropriate screening tests.

Learn more about what to expect when having common tests, procedures, and scans.

Find out more about common terms used during a diagnosis of cancer.

What is the treatment for medullary thyroid cancer that has spread to the lymph nodes of the neck or beyond?

Surgical resection (removal) of lymph nodes in the neck and upper chest (performed by a surgeon experienced with this technique) can result in cure in 10% to 20% of cases where there is spread to neck lymph nodes.

Recent studies of drugs that target the RET gene have shown promise for the treatment of medullary thyroid carcinoma that has spread to liver or other sites. While these studies are promising, these medications are only available through participation in a clinical trial (research study). For more information on treatment options, read the guide to thyroid cancer.

What are the risks associated with pheochromocytoma?

Pheochromocytoma associated with MEN2 is almost always a benign tumor. However, such tumors commonly produce abnormally high levels of adrenalin and noradrenalin and may cause high blood pressure, a rapid or irregular heart rate, or cardiac arrest. Medical treatments to prevent these symptoms are highly effective and should be started at the time of diagnosis and before the surgical removal of the tumor. Learn more in the guide to adrenal gland tumors.

What is the typical treatment for a parathyroid tumor?

Increased production of parathyroid hormone by a benign parathyroid tumor can cause hypercalcemia (high blood calcium), kidney stones, and osteoporosis (loss of bone mass). Surgical removal of the parathyroid tumor often cures the problem. More information can be found in the guide to parathyroid tumors.

Questions to ask the doctor

If you are concerned about your risk of thyroid cancer, talk with your doctor. Consider asking the following questions of your doctor:

• What is my risk of developing thyroid cancer?

• What is my risk of developing other types of tumors?

• What can I do to reduce my risk of cancer?

• What are my options for cancer screening?


If you are concerned about your family history and think you or other family members may have MEN2, consider asking the following questions:

• Does my family history increase my risk of developing thyroid cancer?

• Should I meet with a genetic counselor?

• Should I consider genetic testing?


Additional resources

Guide to Thyroid Cancer


The Genetics of Thyroid Cancer


What to Expect When You Meet With a Genetic Counselor


National Cancer Institute

www.cancer.gov


American Cancer Society

www.cancer.org


CancerCare

www.cancercare.org


To find a genetic counselor in your area, ask your doctor or visit these websites:

National Society of Genetic Counselors

www.nsgc.org


National Cancer Institute

www.cancer.gov/search/geneticsservices