1 Lung Carcinoid Tumor What are lung carcinoid tumors? Lung carcinoid tumors (also known as lung carcinoids) are a type of lung cancer, which is a cancer that starts in the lungs. Cancer starts when cells begin to grow out of control. Cells in nearly any part of the body can become cancer, and can spread to other areas of the body. To learn more about how cancers start and spread, see What Is Cancer? Lung carcinoid tumors are uncommon and tend to grow slower than other types of lung cancers. They are made up of special kinds of cells called neuroendocrine cells. To understand lung carcinoid tumors, it helps to know something about the normal structure and function of the lungs, as well as the neuroendocrine system. The lungs The lungs are 2 sponge-like organs in your chest. Your right lung has 3 sections, called lobes. The left lung has 2 lobes. It is smaller because the heart takes up room on that side of the body.
2 When you breathe in, air enters through your mouth and nose and goes into your lungs through the trachea (windpipe). The trachea divides into tubes called the bronchi (singular, bronchus), which enter the lungs and divide into smaller branches called the bronchioles. At the end of the bronchioles are tiny air sacs known as alveoli. Many tiny blood vessels run through the alveoli. They absorb oxygen from the inhaled air into your bloodstream and pass carbon dioxide (a waste product from the body) into the alveoli. This is expelled from the body when you exhale. Taking in oxygen and getting rid of carbon dioxide are your lungs main functions. A thin lining called the pleura surrounds the lungs. The pleura protects your lungs and helps them slide back and forth as they expand and contract during breathing. The space inside the chest that contains the lungs is called the pleural space (or pleural cavity). Below the lungs, a thin, dome-shaped muscle called the diaphragm separates the chest from the abdomen. When you breathe, the diaphragm moves up and down, forcing air in and out of the lungs.
3 The diffuse neuroendocrine system Carcinoid tumors start from cells of the diffuse neuroendocrine system. This system is made up of cells that are like nerve cells in certain ways and like hormone-making endocrine cells in other ways. These cells do not form an actual organ like the adrenal or thyroid glands. Instead, they are scattered throughout the body in organs like the lungs, stomach, and intestines. Neuroendocrine cells make hormones like adrenaline and similar substances. In the lungs, this may help control air flow and blood flow and may help control the growth of other types of lung cells. Neuroendocrine cells may detect the levels of oxygen and carbon dioxide in the air we breathe and then release chemical messages to help the lungs adjust to these changes. People who live at higher altitudes have more lung neuroendocrine cells, probably because there is less oxygen in the air they breathe. Types of lung neuroendocrine tumors Like most cells in your body, lung neuroendocrine cells sometimes go through certain changes that cause them to grow too much and form tumors. These are known as neuroendocrine tumors or neuroendocrine cancers. Carcinoid tumors are one type of neuroendocrine tumor. Neuroendocrine tumors can develop anywhere in the body. Neuroendocrine tumors that begin in the digestive system, another common site for these tumors, are discussed in our documents Gastrointestinal Carcinoid Tumors and Pancreatic Cancer. This document focuses only on carcinoid tumors that start in the lungs. There are 4 types of neuroendocrine lung tumors. Starting with the fastest growing, they are: Small cell lung cancer Large cell neuroendocrine carcinoma Atypical carcinoid tumor Typical carcinoid tumor Small cell lung cancer Small cell lung cancer (SCLC) is one of the fastest growing and spreading of all cancers. It is discussed in Lung Cancer (Small Cell).
4 Large cell neuroendocrine carcinoma Large cell neuroendocrine carcinoma (LCNEC) is a rare cancer. It is a subtype of non-small cell lung cancer (NSCLC). Although it shares some features with SCLC (including a tendency to grow quickly), it is typically treated as a type of NSCLC. For treatment information, see Lung Cancer (Non-Small Cell). Typical and atypical carcinoid tumors The other 2 types of lung neuroendocrine tumors are carcinoids. Typical and atypical carcinoid tumors look different under the microscope. Typical carcinoids tend to grow slowly and only rarely spread beyond the lungs. About 9 out of 10 lung carcinoids are typical carcinoids. Atypical carcinoids grow a little faster and are somewhat more likely to spread to other organs. They have more cells in the process of dividing and look more like a fast-growing tumor. They are much less common than typical carcinoids. Carcinoids are sometimes also classified by where they form in the lung. Central carcinoids form in the walls of large airways (bronchi) near the center of the lungs. Most lung carcinoid tumors are central carcinoids, and nearly all of these are also typical carcinoids. Peripheral carcinoids develop in the smaller airways (bronchioles) toward the outer edges of the lungs. These are more likely than central carcinoids to be atypical, although most peripheral carcinoids are still typical carcinoids. This distinction is important because the tumor s location can affect which symptoms a patient has (see the section Signs and symptoms of lung carcinoid tumors? ) and may also affect how the tumor is treated. What are the key statistics about lung carcinoid tumors? About 1% to 2% of all lung cancers are carcinoids. There are about 4,000 newly diagnosed lung carcinoid tumors in the United States each year. Carcinoid tumors actually develop more often in the digestive tract than in the lungs. Only about 3 out of 10 carcinoid tumors start in the lungs. Lung carcinoids tend to be diagnosed in people who are slightly younger than the typical age of people with other types of lung cancer. The average age at diagnosis is around 60 years.
5 Information on survival rates for lung carcinoids can be found in the section Survival rates for lung carcinoid tumors. What are the risk factors for lung carcinoid tumors? A risk factor is anything that affects your chance of getting a disease such as cancer. Different cancers have different risk factors. Some risk factors, like smoking, can be changed. Others, like a person s age or family history, can t be changed. But having a known risk factor, or even several risk factors, does not mean that you will get the disease. And some people who get the disease may have few or no known risk factors. Not much is known about why lung carcinoid tumors develop in some people but not in others. Risk factors for lung carcinoid tumors include: Gender Lung carcinoids occur more often in women than in men. The reasons for this are not known. Race/ethnicity Lung carcinoids are more common in whites than in African Americans, Asian Americans, or Hispanics/Latinos. Age These tumors are usually found in people about 60 years old, which is slightly younger than the average age for other types of lung cancer. But carcinoids can occur in people of almost any age. Although it s rare, lung carcinoid tumors are sometimes even found in children. Multiple endocrine neoplasia type 1 People with multiple endocrine neoplasia type 1 (MEN1), an inherited syndrome, are at high risk for tumors in certain endocrine organs, such as the pancreas and the pituitary and parathyroid glands. These people also seem to be at increased risk for lung carcinoid tumors. Family history Most people with lung carcinoid tumors do not have a family history of this type of cancer, but having others in your family who had lung carcinoid tumors can increase your risk. In
6 rare cases, several family members have been diagnosed with this cancer. The overall risk is still low because this cancer is so uncommon. Tobacco smoke Typical lung carcinoid tumors do not seem to be linked with smoking or with any known chemicals in the environment or workplace. But some studies have found that atypical lung carcinoids may be more common in people who smoke. Do we know what causes lung carcinoid tumors? Not much is known about what causes lung carcinoid tumors. Researchers have learned a lot about how certain risk factors like cancer-causing chemicals or radiation can cause changes in lung cells that lead to carcinomas, the more common type of lung cancer. But these factors are not thought to play a large role in the development of lung carcinoid tumors. Carcinoid tumors probably develop from tiny clusters of neuroendocrine cells called carcinoid tumorlets in the lung airways. Tumorlets are sometimes found unexpectedly in lung biopsies done to treat or diagnose other conditions. Under the microscope, tumorlets resemble carcinoid tumors, except that they are much smaller less than 5 mm (about ¼ inch) across. Most tumorlets never grow any bigger, but some may eventually become carcinoid tumors. Researchers have found some common changes in chromosomes and genes inside lung carcinoid tumor cells, which might affect how these cells function. But it is still not clear exactly how these changes cause carcinoid tumorlets to develop from lung neuroendocrine cells or how they might cause tumorlets to grow to become carcinoid tumors. Can lung carcinoid tumors be prevented? Because we do not yet know what causes most lung carcinoid tumors, it is not possible to know how to prevent them. Smoking has been linked with an increased risk of atypical carcinoids in some studies, so quitting (or not starting) might reduce a person s risk. Can lung carcinoid tumors be found early? Lung carcinoid tumors are not common, and there are no widely recommended screening tests for these tumors in most people. (Screening is testing for cancer in people without any symptoms.)
7 People with multiple endocrine neoplasia type 1 (MEN1) are at increased risk for these tumors, and some doctors recommend they have computed tomography (CT) scans of the chest every 3 years starting when they are age 20. Because carcinoid tumors usually grow and spread slowly, most are found at an early or localized stage, even if they have been causing symptoms for some time. Many patients with peripheral carcinoid tumors or with small central carcinoid tumors have no symptoms. Carcinoids that are not causing symptoms often are found on a chest x-ray or CT scan done for other reasons. Signs and symptoms of lung carcinoid tumors About 2 of 3 people with carcinoid tumors will have signs or symptoms that will lead to the diagnosis of the disease. But because carcinoids tend to grow slowly, they may not cause symptoms for several years in some people, or they may be found by medical tests done for other reasons. Central carcinoids Most carcinoid tumors start in the large bronchial tubes leading into the lung. Symptoms can include: Cough, which can sometimes be bloody Wheezing Shortness of breath Chest pain, especially when taking deep breaths Large carcinoids can cause partial or complete blockage of an air passage, which can lead to pneumonia (an infection in the lung). Sometimes a doctor may suspect a tumor only after treatment with antibiotics doesn t cure the pneumonia. Peripheral carcinoids Some tumors start in the smaller airways toward the outer edges of the lungs. They rarely cause any symptoms unless there are so many of them that they cause trouble breathing. Usually they are found as a spot on a chest x-ray or CT scan that is done for an unrelated problem.
8 Symptoms caused by hormones from the tumor Some carcinoid tumors can make hormone-like substances that are released into the bloodstream. Lung carcinoids do this far less often than gastrointestinal carcinoid tumors. Carcinoid syndrome: Rarely, lung carcinoid tumors release enough hormone-like substances into the bloodstream to cause symptoms. This results in the carcinoid syndrome. Symptoms can include Facial flushing (redness and warm feeling) Diarrhea Wheezing Fast heartbeat. Many patients find that stress, heavy exercise, and drinking alcohol can bring on these symptoms or make them worse. Over a long time, these hormone-like substances can damage heart valves, causing: Shortness of breath Weakness Heart murmur (an abnormal heart sound) Cushing syndrome: In rare cases, lung carcinoid tumors may make a hormone called ACTH. This causes the adrenal glands to make too much cortisol (a steroid hormone) and other hormones. This can lead to: Weight gain Easy bruising Weakness Drowsiness High blood sugar (or even diabetes) High blood pressure Increased body and facial hair The symptoms and signs above may be caused by lung carcinoid tumors, but they can also be caused by other conditions. Still, if you have any of these problems, it s important to see your doctor so the cause can be found and treated, if needed.
9 How are lung carcinoid tumors diagnosed? Certain signs and symptoms might suggest that a person could have a lung carcinoid tumor, but tests are needed to confirm the diagnosis. Medical history and physical exam If you have any signs or symptoms that suggest you might have a lung carcinoid tumor (or another type of lung tumor), your doctor will take a complete medical history, including your family history, to learn about your symptoms and possible risk factors. A physical exam can give your doctor information about your general health, possible signs of lung carcinoid tumor, and other health problems. During your exam, your doctor will pay close attention to your chest and lungs. If your symptoms or the results of the exam suggest you might have a lung carcinoid tumor (or another type of tumor), more tests will be done. These might include imaging tests, lab tests, and other procedures. Imaging tests Imaging tests use x-rays, radioactive particles, or other means to create pictures of the inside of your body. Imaging tests are done for a number of reasons, including to help find a suspicious area that might be cancer, to learn how far cancer may have spread, and to help determine if treatment has been effective. Chest x-ray A chest x-ray is often the first imaging test a doctor orders if a lung problem is suspected. It might be able to show if there is a tumor in the lung. But some carcinoids that are small or are in places where they are covered by other organs in the chest may not show up on a chest x-ray. If your doctor is still suspicious or if something is seen on the chest x-ray, a CT scan may be ordered. Computed tomography (CT) scan A CT (or CAT) scan is more likely to show small lung tumors than routine chest x-rays. A CT scan can also provide precise information about the size, shape, and position of any lung tumors and can help find enlarged lymph nodes that might contain cancer that has spread from the lung. The CT scan uses x-rays to produce detailed cross-sectional images of your body. Instead of taking one picture, like a regular x-ray, a CT scanner takes many pictures as it rotates around
10 you while you are lying on a narrow platform. A computer then combines these into images showing slices of the part of your body being studied. Before any pictures are taken, you may be asked to drink 1 to 2 pints of a liquid called oral contrast. This helps outline the intestine so that certain areas are not mistaken for tumors. This is not needed if the CT scan is only looking at the chest and lungs. You may also receive an IV (intravenous) line through which a different kind of contrast dye (IV contrast) is injected. This helps better outline structures in your body. The injection can cause some flushing (redness and warm feeling). Some people are allergic and get hives or, rarely, more serious reactions like trouble breathing and low blood pressure. Be sure to tell the doctor if you have any allergies or have ever had a reaction to any contrast material used for x-rays. A CT scanner has been described as a large donut, with a narrow table that slides in and out of the middle opening. You will need to lie still on the table while the scan is being done. CT scans take longer than regular x-rays, and you might feel a bit confined by the ring while the pictures are being taken. CT scans can be used in several ways: To help determine the exact location and extent of the tumors. To stage a cancer (determining the extent of its spread). This can help to determine if surgery is a good treatment option. To guide a biopsy needle precisely into a suspected tumor. For this procedure, called a CT-guided needle biopsy, you stay on the CT scanning table while the doctor advances a biopsy needle through the skin and toward the mass. CT scans are repeated until the needle is within the mass. A biopsy sample is then removed and looked at under a microscope. To see how effective treatment has been. Radionuclide scans Scans using small amounts of radioactivity and special cameras may be helpful in looking for carcinoid tumors. They can help find tumors or look for areas of cancer spread if doctors aren t sure where they are in the body. Somatostatin receptor scintigraphy: The most common scan is somatostatin receptor scintigraphy (SRS), also known as the OctreoScan. It uses a drug called octreotide bound to radioactive indium-111. Octreotide is a hormone-like substance that attaches to carcinoid cells. A small amount is injected into a vein. It travels through the blood and is attracted to carcinoid tumors. A few hours after the injection, a special camera can be used to show where the radioactivity has collected in the body. More scans may be done in the following few days as well. Along with showing where tumors are, this test can help tell whether treatment with certain drugs such as octreotide and lanreotide is likely to be helpful.
11 I-131 MIBG scan: This test is used less often. It uses a chemical called MIBG attached to radioactive iodine (I-131). This substance is injected into a vein, and the body is scanned several hours or days later with a special camera to look for areas that picked up the radioactivity. These would most likely be carcinoid tumors, but other kinds of neuroendocrine tumors will also pick up this chemical. Positron emission tomography (PET) scan: For most types of cancer, PET scans use a form of radioactive glucose (sugar) to find tumors. But this type of PET scan is not very useful in finding carcinoid tumors. Instead, PET scanning for carcinoid tumors usually uses a radioactive form of 5-hydroxytryptophan, a chemical that is taken up and used by carcinoid cells. A special camera can detect the radioactivity. The usefulness of this test for lung carcinoid tumors is still being studied. This special type of PET scan is not available in every hospital. Sputum cytology Even if an imaging test such as a chest x-ray or CT scan shows a mass, it s often hard for doctors to tell if the mass is a carcinoid tumor, another type of lung cancer, or an area of infection. Tests may be needed to get a sample of the abnormal cells to be looked at under a microscope. One way to do this is called sputum cytology. A sample of sputum (mucus you cough up from the lungs) is looked at under a microscope to see if it contains cancer cells. The best way to do this is to get samples taken early in the morning, 3 days in a row. This test is not as good at finding lung carcinoids as it is at finding other types of lung cancers. Biopsy In many cases, the only way to know for sure if a person has some type of lung cancer is to remove cells from the tumor and look at them under a microscope. This procedure is called a biopsy. There are several ways to take a sample from a lung tumor. Bronchoscopy and biopsy This approach is used to view and sample tumors in large airways. The doctor passes a long, thin, flexible, fiber-optic tube called a bronchoscope down the throat and through your windpipe and bronchi to look at the lining of the lung s main airways. Your mouth and throat are sprayed first with a numbing medicine. You may also be given medicine through an intravenous (IV) line to make you feel relaxed. If a tumor is found, the doctor can take biopsies (small samples of the tumor) through the tube. The doctor can also sample cells from the lining of the airways by wiping a tiny brush over the surface of the tumor (bronchial brushing) or by rinsing the airways with sterile
12 saltwater and then collecting it (bronchial washing). Brushing and washing samples are sometimes helpful additions to the bronchial biopsy, but they are not as helpful in diagnosing carcinoids as they are with other lung cancers. An advantage of this type of biopsy is that no surgery or hospital stay is needed, and you are ready to return home within hours. A disadvantage is that this type of biopsy may not always be able to remove enough to be certain that a tumor is a carcinoid. But with recent advances in the lab testing of lung tumors, doctors can usually make an accurate diagnosis even with very small samples. Bleeding from a carcinoid tumor after a biopsy is rare but it can be serious. If bleeding becomes a problem, doctors can inject drugs through the bronchoscope into the tumor to narrow its blood vessels, or they can seal off the bleeding vessels with a laser aimed through the bronchoscope. Endobronchial ultrasonography (EBUS) and biopsy If a CT scan shows lymph nodes are enlarged on either side of the trachea or in the area just below where the trachea divides, this test can be used to biopsy these nodes to see if they contain cancer. Ultrasound is a type of imaging test that uses sound waves to create pictures of the inside of your body. For this test, a small, microphone-like instrument called a transducer gives off sound waves and picks up the echoes as they bounce off body tissues. The echoes are converted by a computer into a black and white image on a computer screen. For endobronchial ultrasound, a bronchoscope is fitted with an ultrasound transducer at its tip and is passed down into the windpipe. This is done with numbing medicine (local anesthesia) and light sedation. The transducer can be pointed in different directions to look at lymph nodes and other structures in the mediastinum (the area between the lungs). A hollow needle can be passed through the bronchoscope to get biopsy samples of enlarged lymph nodes or other abnormal areas. The samples are then sent to a lab to be looked at under a microscope. Needle biopsies Doctors can often use a hollow needle to get a small sample from a suspicious area (mass). An advantage of needle biopsies is that they don t require a surgical incision, but in some cases they might not get enough of a sample to make a diagnosis. There are two types of needle biopsies, based on the type of needle used: In a fine needle aspiration (FNA) biopsy, the doctor uses a syringe with a very thin, hollow needle (thinner than the ones used for blood tests) to withdraw (aspirate) cells and small fragments of tissue.
13 In a core biopsy, a larger needle is used to remove one or more small cylinders (cores) of tissue. Core biopsies provide a larger sample than FNA biopsies. If the suspected tumor is in the outer part of the lungs, either kind of biopsy needle can be inserted through the skin on the chest wall. This is called a transthoracic needle biopsy. The area where the needle is to be inserted may be numbed with local anesthesia first. The doctor then guides the needle into the area while looking at the lungs with either fluoroscopy (which is like an x-ray, but the image is shown on a screen rather than on film) or CT scans. Unlike fluoroscopy, CT doesn t give a constant picture, so the needle is inserted toward the mass, a CT image is taken, and the direction of the needle is guided based on the image. This is repeated a few times until the needle is in the mass. A possible complication of this procedure is that air may leak out of the lung at the biopsy site and into the space between the lung and the chest wall. This can cause part of the lung to collapse and could cause trouble breathing. This complication is called a pneumothorax. If the air leak is minimal, it often gets better without any treatment. A large pneumothorax is treated by putting a small tube into the chest space and sucking out the air over a day or two, after which it usually heals on its own. An FNA biopsy may also be done to check for cancer in the lymph nodes between the lungs. This can be done in two ways: Transtracheal FNA or transbronchial FNA is done by passing the needle through the wall of the windpipe (trachea) or bronchi (the large airways leading into the lungs) during bronchoscopy or EBUS (already described above). In some cases an FNA biopsy is done during endoscopic esophageal ultrasound (which is similar to EBUS, except that the scope is passed down the esophagus instead of the windpipe) by passing the needle through the wall of the esophagus. Surgical biopsies In some cases, the types of biopsies above can t remove enough tissue to identify the type of tumor, and your doctor may need to do surgery to get a biopsy sample. Different types of operations may be used. They are most often done in the operating room while you are under general anesthesia (in a deep sleep). Thoracotomy: For a thoracotomy, the surgeon makes an incision (cut) in the chest wall between the ribs to get to the lungs and to the space between the lungs and the chest wall. In some cases if the doctor strongly suspects a carcinoid or some other type of lung cancer, they may do a thoracotomy and remove the entire tumor without first doing a biopsy. Thoracoscopy: This procedure is also used to look at the space between the lungs and the chest wall, but it does not require a long incision like a thoracotomy. The doctor inserts a thin, lighted scope with a small video camera on the end through a small cut made in the chest wall to look at the outside of the lungs and the space between the lungs and the chest
14 wall. (Sometimes more than one cut is made.) Using this scope, the doctor can see potential areas of cancer and remove small pieces of tissue to look at under the microscope. Thoracoscopy can also be used to sample lymph nodes and fluid and find out if a tumor is growing into nearby tissues or organs. Mediastinoscopy: This procedure may be done if imaging tests such as a CT scan suggest that the cancer may have spread to the lymph nodes in the mediastinum (the space between the lungs). A small cut is made in the front of the neck and a thin, hollow, lighted tube is inserted behind the sternum (breast bone) and in front of the windpipe to look at the area. Instruments can be passed through this tube to take tissue samples from the lymph nodes along the windpipe and the bronchi. Blood and urine tests Because carcinoid tumors can secrete hormone-like chemicals into the blood, these tumors can sometimes be found with blood or urine tests. This is especially true if you have symptoms of the carcinoid syndrome, which is caused by excess levels of these chemicals in the blood. Serotonin is made by some carcinoid tumors, and probably causes some of the symptoms. It is broken down by the body into 5-hydroxyindoleacetic acid (5-HIAA), which is released into the urine. A common test to look for carcinoid syndrome measures the levels of 5-HIAA in a urine sample collected over 24 hours. Measuring the serotonin levels in the blood or urine may also give useful information. These tests can help diagnose some carcinoid tumors, but they are not always accurate. Some other medical conditions, as well as foods and medicines, can affect the results, and some carcinoid tumors may not release enough of these substances to give a positive test result. Other tests used to look for carcinoids include blood tests for chromogranin A (CgA), neuron-specific enolase (NSE), cortisol, and substance P. Depending on where the tumor might be located and on the patient s symptoms, doctors may do other blood tests as well. These tests are less likely to be helpful with lung carcinoid tumors than with carcinoid tumors that start elsewhere in the body. Pulmonary function tests If a lung carcinoid is found, pulmonary function tests (PFTs) are often done to see how well your lungs are working. This is especially important if surgery might be used to treat the cancer. Because surgery will remove part or all of the lung, it s important to know how well your lungs are working. These tests can give the surgeon an idea of whether surgery is a good option, and if so, how much lung can be removed safely. There are a few different types of PFTs, but they all basically have you breathe in and out through a tube that is connected to a machine that measures airflow.
15 How are lung carcinoid tumors staged? The stage of a cancer describes how far it has spread. Your treatment and prognosis (outlook) depend, to a large extent, on the cancer s stage. The stage of the cancer is based on the results of the physical exam, imaging tests (CT scan, PET scan, etc.), and other tests, which are described in the section How are lung carcinoid tumors diagnosed? The TNM staging system A staging system is a standard way for the cancer care team to sum up how large a cancer is and how far it has spread. Lung carcinoid tumors are staged using the same system used to stage other types of lung cancer the American Joint Committee on Cancer (AJCC) TNM staging system. The TNM system describes 3 key pieces of information: T indicates the size of the main (primary) tumor and whether it has grown into nearby areas. N describes the spread of cancer to nearby (regional) lymph nodes. Lymph nodes are small bean-shaped collections of immune system cells to which cancers often spread. M indicates whether the cancer has spread (metastasized) to other organs of the body. (The most common site is the liver.) Numbers or letters appear after T, N, and M to provide more details about each of these factors. The numbers 0 through 4 indicate increasing severity. The TNM staging system is complex and can be hard for patients (and even some doctors) to understand. If you have any questions about the stage of your cancer, ask your doctor to explain it to you. T categories TX: The main (primary) tumor can t be assessed, or cancer cells were seen on sputum cytology or bronchial washings but no tumor can be found. T0: There is no evidence of a primary tumor. Tis: The cancer is found only in the top layers of cells lining the air passages. It has not invaded into deeper lung tissues. This stage is also known as carcinoma in situ. T1: The tumor is no larger than 3 centimeters (cm) slightly less than 1¼ inches across. It has not reached the membranes that surround the lungs (visceral pleura), and it does not affect the main branches of the bronchi.
16 T1a: The tumor is 2 cm (about 4/5 of an inch) or less across T1b: The tumor is larger than 2 cm but not larger than 3 cm across T2: The tumor has 1 or more of the following features: It is larger than 3 cm but not larger than 7 cm across. o T2a: The tumor is 5 cm or less across but still larger than 3 cm o T2b: The tumor is larger than 5 cm across but not larger than 7 cm It involves a main bronchus, but is not closer than 2 cm (about ¾ inch) to the carina (the point where the windpipe splits into the left and right main bronchi). It has grown into the membranes that surround the lungs (visceral pleura). The tumor partially clogs the airways, but this has not caused the entire lung to collapse or pneumonia to develop. T3: The tumor has 1 or more of the following features: It is larger than 7 cm across. It has grown into the chest wall, the breathing muscle that separates the chest from the abdomen (diaphragm), the membranes surrounding the space between the lungs (mediastinal pleura), or membranes of the sac surrounding the heart (parietal pericardium). It invades a main bronchus and is closer than 2 cm (about ¾ inch) to the carina, but it does not involve the carina itself. It has grown into the airways enough to cause an entire lung to collapse or to cause pneumonia in the entire lung. Two or more separate tumor nodules are present in the same lobe of a lung T4: The cancer has 1 or more of the following features: A tumor of any size has grown into the space between the lungs (mediastinum), the heart, the large blood vessels near the heart (such as the aorta), the windpipe, the esophagus (tube connecting the throat to the stomach), the backbone, or the carina. Two or more separate tumor nodules are present in different lobes of the same lung.
17 N categories NX: Nearby lymph nodes cannot be assessed. N0: The cancer has not spread to nearby lymph nodes. N1: The cancer has spread to lymph nodes within the lung and/or around the area where the bronchus enters the lung (hilar lymph nodes). Affected lymph nodes are on the same side as the primary tumor(s). N2: The cancer has spread to lymph nodes around the carina (the point where the windpipe splits into the left and right bronchi) or to lymph nodes in the space between the lungs (mediastinum) on the same side as the primary tumor. N3: The cancer has spread to lymph nodes near the collarbone on either side, and/or spread to hilar or mediastinal lymph nodes on the side opposite the primary tumor. M categories M0: The cancer has not spread to distant organs or areas. This includes the other lung, lymph nodes farther away than those mentioned in the N stages above, and other organs or tissues such as the liver, bones, or brain. M1a: Any of the following:
18 The cancer has spread to the other lung. Cancer cells are found in the fluid around the lung (called a malignant pleural effusion). Cancer cells are found in the fluid around the heart (called a malignant pericardial effusion). M1b: The cancer has spread to distant lymph nodes or to organs such as the liver, bones, or brain. Stage grouping Once the T, N, and M categories have been determined, this information is combined to assign an overall stage of 0, I, II, III, or IV. This process is called stage grouping. Some stages are subdivided into A and B. The stages identify tumors that have a similar outlook (prognosis) and so they are treated in a similar way. Patients with lower stage numbers tend to have a better outlook. Occult (hidden) cancer TX, N0, M0: Cancer cells are seen in a sample of sputum or other lung fluids, but the cancer isn t found with other tests, so its location can t be determined. Stage 0 Tis, N0, M0: The cancer is found only in the top layers of cells lining the air passages. It has not invaded deeper into other lung tissues and has not spread to lymph nodes or distant sites. Stage IA T1, N0, M0: The cancer is no larger than 3 cm across, has not reached the membranes that surround the lungs, and does not affect the main branches of the bronchi. It has not spread to lymph nodes or distant sites. Stage IB T2a, N0, M0: The cancer has 1 or more of the following features: The main tumor is larger than 3 cm across but not larger than 5 cm. The tumor involves a main bronchus, but is not within 2 cm of the carina and is not larger than 5 cm. The tumor has grown into the visceral pleura (the membranes surrounding the lungs) and is not larger than 5 cm. The cancer is partially clogging the airways and is not larger than 5 cm. The cancer has not spread to lymph nodes or distant sites.
19 Stage IIA Three main combinations of categories make up this stage. T1, N1, M0: The cancer is no larger than 3 cm across, has not grown into the membranes that surround the lungs, and does not affect the main branches of the bronchi. It has spread to lymph nodes within the lung and/or around the area where the bronchus enters the lung (hilar lymph nodes). These lymph nodes are on the same side as the cancer. It has not spread to distant sites. OR T2a, N1, M0: The cancer has 1 or more of the following features: The main tumor is larger than 3 cm across but not larger than 5 cm. The tumor has grown into a main bronchus, but is not within 2 cm of the carina and is not larger than 5 cm. The tumor has grown into the visceral pleura (the membranes surrounding the lungs) and is not larger than 5 cm. The cancer is partially clogging the airways and is not larger than 5 cm. The cancer has also spread to lymph nodes within the lung and/or around the area where the bronchus enters the lung (hilar lymph nodes). These lymph nodes are on the same side as the cancer. It has not spread to distant sites. OR T2b, N0, M0: The cancer has 1 or more of the following features: The main tumor is larger than 5 cm across but not larger than 7 cm. The tumor has grown into a main bronchus and is between 5 and 7 cm across, but it is not within 2 cm of the carina. The tumor has grown into the visceral pleura (the membranes surrounding the lungs) and is between 5 and 7 cm across. The cancer is partially clogging the airways and is between 5 and 7 cm across. The cancer has not spread to lymph nodes or distant sites. Stage IIB Two combinations of categories make up this stage. T2b, N1, M0: The cancer has 1 or more of the following features:
20 The main tumor is larger than 5 cm across but not larger than 7 cm. The tumor has grown into a main bronchus and is between 5 and 7 cm across, but it is not within 2 cm of the carina. The tumor has grown into the visceral pleura (the membranes surrounding the lungs) and is between 5 and 7 cm across. The tumor is partially clogging the airways and is between 5 and 7 cm across. The cancer has also spread to lymph nodes within the lung and/or around the area where the bronchus enters the lung (hilar lymph nodes). These lymph nodes are on the same side as the cancer. It has not spread to distant sites. OR T3, N0, M0: The main tumor has 1 or more of the following features: It is larger than 7 cm across. It has grown into the chest wall, the breathing muscle that separates the chest from the abdomen (diaphragm), the membranes surrounding the space between the lungs (mediastinal pleura), or membranes of the sac surrounding the heart (parietal pericardium). It invades a main bronchus and is closer than 2 cm (about ¾ inch) to the carina, but it does not involve the carina itself. It has grown into the airways enough to cause an entire lung to collapse or to cause pneumonia in the entire lung. Two or more separate tumor nodules are present in the same lobe of a lung. The cancer has not spread to lymph nodes or distant sites. Stage IIIA Three main combinations of categories make up this stage. T1 to T3, N2, M0: The tumor can be any size or have any of the following features: The tumor invades a main bronchus without growing into the carina. The tumor has grown into the visceral pleura (the membranes surrounding the lungs). The tumor is partially clogging the airways, or it has grown into the airways enough to cause an entire lung to collapse or to cause pneumonia in the entire lung. It has grown into the chest wall, the breathing muscle that separates the chest from the abdomen (diaphragm), the membranes surrounding the space between the lungs
21 (mediastinal pleura), or membranes of the sac surrounding the heart (parietal pericardium). Two or more separate tumor nodules are present in the same lobe of a lung. The cancer has also spread to lymph nodes around the carina (the point where the windpipe splits into the left and right bronchi) or in the space between the lungs (mediastinum). These lymph nodes are on the same side as the main lung tumor. The cancer has not spread to distant sites. OR T3, N1, M0: The tumor has 1 or more of the following features: It is larger than 7 cm across. It has grown into the chest wall, the breathing muscle that separates the chest from the abdomen (diaphragm), the membranes surrounding the space between the lungs (mediastinal pleura), or membranes of the sac surrounding the heart (parietal pericardium). It invades a main bronchus and is closer than 2 cm to the carina, but it has not grown into the carina itself. Two or more separate tumor nodules are present in the same lobe of a lung. It has grown into the airways enough to cause an entire lung to collapse or to cause pneumonia in the entire lung. The cancer has also spread to lymph nodes within the lung and/or around the area where the bronchus enters the lung (hilar lymph nodes). These lymph nodes are on the same side as the cancer. It has not spread to distant sites. OR T4, N0 or N1, M0: The cancer has 1 or more of the following features: A tumor of any size has grown into the space between the lungs (mediastinum), the heart, the large blood vessels near the heart (such as the aorta), the windpipe, the esophagus (tube connecting the throat to the stomach), the backbone, or the carina. Two or more separate tumor nodules are present in different lobes of the same lung. The cancer may or may not have spread to lymph nodes within the lung and/or around the area where the bronchus enters the lung (hilar lymph nodes). Any affected lymph nodes are on the same side as the cancer. It has not spread to distant sites. Stage IIIB
22 Two combinations of categories make up this stage. Any T, N3, M0: The cancer can be of any size. It may or may not have grown into nearby structures or caused pneumonia or lung collapse. It has spread to lymph nodes near the collarbone on either side, and/or has spread to hilar or mediastinal lymph nodes on the side opposite the primary tumor. The cancer has not spread to distant sites. OR T4, N2, M0: The cancer has 1 or more of the following features: A tumor of any size has grown into the space between the lungs (mediastinum), the heart, the large blood vessels near the heart (such as the aorta), the windpipe, the esophagus (tube connecting the throat to the stomach), the backbone, or the carina. Two or more separate tumor nodules are present in different lobes of the same lung. The cancer has also spread to lymph nodes around the carina or in the space between the lungs (mediastinum). Affected lymph nodes are on the same side as the main lung tumor. It has not spread to distant sites. Stage IV Two combinations of categories make up this stage. Any T, any N, M1a: The cancer can be any size and may or may not have grown into nearby structures or reached nearby lymph nodes. In addition, any of the following is true: OR The cancer has spread to the other lung. Cancer cells are found in the fluid around the lung (called a malignant pleural effusion). Cancer cells are found in the fluid around the heart (called a malignant pericardial effusion). Any T, any N, M1b: The cancer can be any size and may or may not have grown into nearby structures or reached nearby lymph nodes. It has spread to distant sites, such as the liver. Survival rates for lung carcinoid tumors Survival rates are often used by doctors as a standard way of discussing a person s prognosis (outlook). Some patients with cancer may want to know the survival statistics for people in similar situations, while others may not find the numbers helpful, or may even not want to know them. If you do not want to read about survival rates for lung carcinoid tumors, stop reading here and skip to another section.
23 The 5-year survival rate refers to the percentage of patients who live at least 5 years after their cancer is diagnosed. Of course, many people live much longer than 5 years (and many are cured). To get 5-year survival rates, doctors have to look at people who were treated at least 5 years ago. Improvements in treatment since then may result in a better outlook for people now being diagnosed. Overall, the 5-year survival rate for patients with typical lung carcinoids is around 85% to 90%, and the 5-year survival rate for patients with atypical lung carcinoids is around 50% to 70%. These ranges reflect different survival rates quoted by several different studies in medical journals. Lung carcinoids are uncommon tumors, so it s hard to get accurate, up-to-date survival statistics for these cancers based on stage. The numbers below come from a study of more than 1,400 people in the United States who were diagnosed with lung carcinoid tumors between 1990 and 2002 and were treated with surgery. They include some people who died from causes other than their cancer. Stage 5-year Survival Rate I 93% II 85% III 75% IV 57% These numbers include people with both typical and atypical carcinoids, but survival rates would be expected to be slightly better for typical carcinoids and not as good overall for atypical carcinoids. Survival rates are often based on previous outcomes of large numbers of people who had the disease, but they cannot predict what will happen in any particular person s case. Many factors can affect a person s outlook, such as the type of treatment used, how well the cancer responds to treatment, and their general health. Your doctor is familiar with your situation and can probably tell you how the numbers above might apply to you. Even with carcinoids that appear to have been treated successfully, in a small number of cases the cancer can come back (recur) many years later, which is why doctors often advise close follow-up for at least 10 years.
24 How are lung carcinoid tumors treated? This information represents the views of the doctors and nurses serving on the American Cancer Society s Cancer Information Database Editorial Board. These views are based on their interpretation of studies published in medical journals, as well as their own professional experience. The treatment information in this document is not official policy of the Society and is not intended as medical advice to replace the expertise and judgment of your cancer care team. It is intended to help you and your family make informed decisions, together with your doctor. Your doctor may have reasons for suggesting a treatment plan different from these general treatment options. Don t hesitate to ask him or her questions about your treatment options. Making treatment decisions for lung carcinoid tumors After the lung carcinoid tumor is found and staged, your cancer care team will discuss your treatment options with you. The main factors in selecting a treatment are the type of carcinoid, the size and location of the tumor, whether it has spread to lymph nodes or other organs, and if you have any other serious medical conditions. Based on these factors, the main treatment options for people with lung carcinoid tumors can include: Surgery Chemotherapy Other drug treatments Radiation therapy These treatments might be used alone or in different combinations, depending on the type and extent of the disease. Selecting a treatment plan is an important decision, and you should take the time to think about all of your choices. Be sure to discuss all of your treatment options as well as their possible side effects with your doctors to help make the decision that best fits your needs. (See the section What should you ask your doctor about lung carcinoid tumors? for some questions to ask.) Seeking a second opinion is often a good idea if time permits. It can give you more information and help you feel more confident about the treatment plan you choose. You may have different types of doctors on your treatment team, depending on the stage of your cancer and your treatment options. These doctors may include: A thoracic surgeon: a doctor who treats diseases of the lungs and chest with surgery A medical oncologist: a doctor who treats cancer with medicines such as chemotherapy A pulmonologist: a doctor who specializes in medical treatment of diseases of the lungs
25 A radiation oncologist: a doctor who treats cancer with radiation therapy Many other specialists might be part of your treatment team as well, including physician assistants (PAs), nurse practitioners (NPs), nurses, nutrition specialists, social workers, and other health professionals. To learn more about who may be on your cancer care team, see Health Professionals Associated With Cancer Care. Surgery to treat lung carcinoid tumors Surgery is the main treatment for lung carcinoid tumors whenever possible. If the tumor hasn t spread, it can often be cured by surgery alone. Types of lung surgery Different operations can be used to treat (and possibly cure) lung carcinoid tumors. These operations require general anesthesia (where you are in a deep sleep) and are usually done through a surgical incision between the ribs in the side of the chest (called a thoracotomy). Pneumonectomy: An entire lung is removed in this surgery. Lobectomy: An entire section (lobe) of a lung is removed in this surgery. Segmentectomy or wedge resection: Part of a lobe is removed in this surgery. Sleeve resection, another type of operation, may be used to treat some cancers in large airways in the lungs. If you think of the large airway with a tumor as similar to the sleeve of a shirt with a stain an inch or 2 above the wrist, this surgery would be like cutting across the sleeve above and below the stain and sewing the cuff back onto the shortened sleeve. A surgeon may be able to do this operation instead of a pneumonectomy to preserve more lung function. With any of these operations, nearby lymph nodes are also removed to look for possible spread of the cancer. The type of operation your doctor recommends depends on the size and location of the tumor and on how well your lungs are functioning. People whose lungs are healthier can withstand having more lung tissue removed. When you wake up from surgery, you will have a tube (or tubes) coming out of your chest and attached to a special canister to allow excess fluid and air to drain out. The tube(s) will be removed once the fluid drainage and air leak subside. Generally, you will need to spend 5 to 7 days in the hospital after the surgery.
26 Lymph node sampling With any of these operations, lymph nodes near the lungs are usually removed to look for possible spread of the cancer. This is important because the carcinoid might have spread to lymph nodes by the time it is diagnosed. (This risk is higher for atypical carcinoids than for typical carcinoids.) If the lymph nodes containing cancer are not removed, it will increase the risk of the carcinoid tumor spreading even farther, to other organs. If this happens, you may no longer be able to be cured by surgery. Checking for cancer cells in the lymph nodes can also provide some indication of your risk of having the cancer come back. Video-assisted thoracic surgery (VATS) This is a less invasive type of surgery for some cancers in the lungs. During this operation, a thin, rigid tube with a tiny video camera on the end is placed through a small cut in the side of the chest to help the surgeon see inside the chest. One or two other small cuts are created in the skin, and long instruments are passed though these cuts to do the same operation that would be done using an open approach (thoracotomy). Because only small incisions are needed, there is less pain after the surgery and a shorter hospital stay usually around 4 to 5 days. Most experts recommend that only smaller tumors near the outside of the lung be treated this way. The cure rate after this surgery seems to be the same as with surgery done with a larger incision. But it is important that the surgeon doing this operation be experienced because it requires a great deal of technical skill. Possible risks and side effects of lung surgery Possible complications depend on the extent of the surgery and the person s health beforehand. Serious complications can include excessive bleeding, wound infections, and pneumonia. Lung surgery is a major operation, and recovering from the operation typically takes weeks to months. If the surgery is done through a thoracotomy, the surgeon must spread the ribs to get to the lung, so the area near the incision will hurt for some time after surgery. Your activity will be limited for at least a month. People who have VATS instead of thoracotomy have less pain after surgery and tend to recover more quickly. If your lungs are in good condition (other than the presence of the cancer) you can usually return to normal activities after a lobe or even an entire lung has been removed. If you also have non-cancerous diseases such as emphysema or chronic bronchitis (which are common among heavy smokers), you may become short of breath with activity after surgery.
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