Carcinoid TumorLast Updated: August 03, 2011 This section has been reviewed and approved by the Cancer.Net Editorial Board, 04/11 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. 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. 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 it can also start in 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:
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):
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. Carcinoid tumors make up only 1% of cancers of the gastrointestinal tract, but they make up about 50% of all small intestine cancers. The overall survival rate (the percentage of people who survive after the cancer is detected, excluding those who die from other diseases) for people with a gastrointestinal carcinoid tumor that has not spread is 67%. Each year, an estimated 4,400 adults in the United States are diagnosed with a lung carcinoid tumor. The 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 60%. 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 and may not apply to a single person. 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. Medical Illustrations
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 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 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 and Signs
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.
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. 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.
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.
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 have 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). Your doctor will ask you questions about the symptoms you are experiencing to help find out the cause of the problem, called a diagnosis. This may include how long you’ve been experiencing the symptom(s) and how often. If cancer is diagnosed, relieving symptoms and side effects remains an important part of cancer care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your health care team about symptoms you experience, including any new symptoms or a change in symptoms. 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:
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); 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 measurement of 5-HIAA (see Overview). 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 examined 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 and 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 an 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 help indicate a tumor or the stage of the 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 way to create a picture of the structures inside of your 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. 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, and 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. MRI often produces better resolution of carcinoid tumor than CT scanning. 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 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 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. After these 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 cancer; this is called staging. Learn more about the first steps to take after 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 whether 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. 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. 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. If there is a recurrence, the cancer may need to be staged again (re-staging). Treatment
This section outlines treatments that are the standard of care (the best proven treatments available) for this specific type of cancer. 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 treatment to evaluate whether 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 and Current 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. Learn more about making treatment decisions. Surgery Surgery is the removal of the tumor and surrounding tissue during an operation. Many carcinoid tumors are successfully treated with surgery alone. A surgical oncologist is a doctor who specializes in cancer 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. Procedures to treat liver metastases 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. 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 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 carcinoid tumors, and it is most often used to relieve symptoms (such as pain) from cancer spread to the bone as part of palliative care. 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, usually by stopping the cancer cells’ ability to grow and divide. 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. 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, 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 made either 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 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 normal cells, usually leading to fewer side effects than other cancer medications. 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 under investigation in clinical trials include drugs that interfere with new vessel formation or with specific survival pathways of cancer cells. Learn more in Current Research. 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. A rectal 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 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 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 and radiation therapy may also be offered to help relieve symptoms. Recurrent carcinoid tumor Once your treatment is complete and there is a remission (absence of cancer symptoms; also called “no evidence of disease” or NED), talk with your doctor about the possibility of the cancer returning. Many survivors feel worried or anxious that the cancer will come back. Learn more about coping with this fear. 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. 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 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. Metastatic carcinoid tumor 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. Participation in clinical trials is encouraged. In addition to treatment to slow, stop, or eliminate the cancer (also called disease-directed treatment), an important part of cancer care is relieving a person’s symptoms and side effects. It includes supporting the patient with his or her physical, emotional, and social needs, an approach called palliative or supportive care. People often receive disease-directed therapy and treatment to ease symptoms at the same time. If disease-directed treatment is not successful, this may also be called advanced cancer. This diagnosis is stressful, and it may be difficult to discuss. 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. Learn more about advanced cancer care planning. Find out more about common terms used during cancer treatment. About Clinical Trials
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 people, 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. 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 clinical trials. For specific topics being studied for a carcinoid tumor, learn more in the Current 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. 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 trials ends, and/or if the patient chooses to leave the clinical trial before it ends. 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 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 your 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. Also, be sure to communicate with the doctor about side effects you experience during and after treatment. Care of a patient’s symptoms and side effects is an important part of a person’s overall treatment plan; this is called palliative or supportive care. It helps people with cancer at any stage of illness be as comfortable as possible. Learn more about the most common side effects of cancer and different treatments, along with ways to prevent or control them. Be sure to talk with your doctor 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 psychosocial (emotional and social) effects as well. For many patients, a diagnosis of a carcinoid tumor is stressful and can bring difficult emotions. Patients and their families are encouraged to share their feelings with a member of their health care team who can help with coping strategies. Learn more about the importance of addressing such needs, including concerns about managing the cost of your medical care. A side effect that occurs more than five years after treatment is called a late effect. Treatment of late effects is an important part of survivorship 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 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 the next steps to take in survivorship, including making positive lifestyle changes. Find out more about common terms used after cancer treatment is complete. Current Research
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 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 MEN1 may play a role in an individual’s risk level for carcinoid tumors. 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. Learn more about common statistical terms used in cancer research. To find clinical trials specific to your diagnosis, talk with your doctor or search online clinical trial databases now. Questions to Ask the Doctor
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.
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. |
