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carcinoid-tumor

Carcinoid Tumor

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What is Carcinoid Tumor?

A carcinoid tumor starts in the hormone-producing cells of various organs, mostly in the gastrointestinal tract (such as the stomach and intestines) and the 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 carcinoid tumors 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 described 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% occurs in the liver.

They rarely occur in ovaries, testicles, and other organs.

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 causes high levels of serotonin and chromogranin-A in the blood and high levels of 5-HIAA (a product of serotonin breakdown) in the urine.

An atypical lung carcinoid tumor 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 it can make 5-HTP.

Symptoms & 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 produced by the tumor can travel throughout the body and cause carcinoid syndrome (see below), a group of symptoms caused by hormones being released by the tumor into the bloodstream. Intestinal carcinoid tumors are most likely to cause symptoms.

People with a gastrointestinal carcinoid tumor may experience the following symptoms or signs. 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 somewhere in the gastrointestinal tract)

Symptoms: lung carcinoid tumor

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

People with a lung carcinoid tumor may experience the following symptoms or signs. 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 a carcinoid tumor 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 and significant fluctuations in blood pressure

Neurosis (a psychological 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 generally occurs after a person has 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 helps raise 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. 

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 a carcinoid tumor 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.

Risk Factors

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

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

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

Family history of multiple endocrine neoplasia type 1. Multiple endocrine neoplasia type 1 (MEN1) is a hereditary condition that increases the risk of developing tumors in 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.

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 spread. This list describes options for diagnosing this type of cancer, and not all tests listed will be used for every person. Your doctor may consider these factors when choosing a diagnostic test:

Age and medical condition

Type of cancer suspected

Signs and symptoms

Previous test results

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

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

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

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 a tumor in the lower section of the digestive system.

Endoscopic ultrasound. An ultrasound uses sound waves to create a picture of the internal organs. This procedure is often done at the same time as an upper endoscopy. 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 the bones. The tracer is injected into a patient’s vein. It collects in areas of the bone and is detected by a special camera. Healthy bone appears gray to the camera, and areas of injury, such as those caused by cancer, appear dark.

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

Barium x-rays. 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) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. A CT scan can also be used to measure the tumor’s size. Additionally, a CT scan is used to see if the tumor has spread to the liver and to detect a carcinoid tumor in the retroperitoneal (the area behind the abdomen) lymph nodes. Sometimes, a contrast medium (a special dye) is injected into a patient’s vein or given orally (by mouth) 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 or given orally (by mouth) to create a clearer picture. MRI often produces clearer images 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 has built up in the body. This procedure is useful for identifying where a carcinoid tumor has spread, especially if it has spread to the liver.

Positron emission tomography (PET) scan. A PET scan is a way to create pictures of organs and tissues inside the body. A small amount of a radioactive 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 a carcinoid tumor grows very slowly, a PET scan may not be as helpful as other tests for diagnosis.

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.

Stages

Staging is a way of describing where the tumor is located, if or where it has spread, and whether it is affecting other parts of the body. Doctors use diagnostic tests to 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 staged similarly to stomach cancer.

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

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

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

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

Lung carcinoid tumor

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

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

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