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lung-carcinoid-tumor

Lung Carcinoid Tumor

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What are lung carcinoid tumors?

Lung carcinoid tumors are an uncommon type of tumor that starts in the lungs. They tend to grow slower than other types of lung cancers. They are made up of special kinds of cells called neuroendocrine cells.

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. 

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: (a) Small cell lung cancer, (b) Large cell neuroendocrine carcinoma, (c) Atypical carcinoid tumor, (d) Typical carcinoid tumor

Small cell lung cancer: Small cell lung cancer (SCLC) is one of the fastest growing and spreading of all cancers.

Large cell neuroendocrine carcinoma: Large cell neuroendocrine carcinoma (LCNEC) is a rare cancer. It is a type of large cell carcinoma, which is actually a type 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. 

Typical and atypical carcinoid tumors

The other 2 types of lung neuroendocrine tumors are carcinoids. The rest of this document will only cover these 2 types of tumors.

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. Seen under a microscope, 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 narrower 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.

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 risk factors don't tell us everything. 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.

Gender: Carcinoids occur more often in women than in men. The reasons for this are not known.

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 is 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 a tendency to develop lung carcinoid tumors can be inherited. In rare cases, several family members have been diagnosed with this cancer. But because this cancer is so uncommon, the risk is still low.

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?

Very little 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 in the lung airways called carcinoid tumorlets. 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 (¼ inch) across.

Most tumorlets never grow any bigger, but some may eventually become carcinoid tumors. Researchers still do not understand how carcinoid tumorlets develop from lung neuroendocrine cells or why tumorlets sometimes 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.)

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 do not cause symptoms often are found on a chest x-ray or CT scan done for other reasons.

How are lung carcinoid tumors diagnosed?

Certain signs and symptoms might suggest that a person may have a lung carcinoid tumor, but tests are needed to confirm the diagnosis.

Signs and symptoms

About 2 out 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 carcinoid tumors: These tumors start in the large bronchial tubes leading into the lung. People with these tumors may have a cough, sometimes with bloody sputum, or they may have wheezing symptoms like asthma. Other possible symptoms include shortness of breath and chest pain, especially when taking deep breaths.

Large carcinoids can cause partial or complete blockage of an air passage, leading to a lung infection called post-obstructive pneumonia. Sometimes a doctor may suspect a tumor only after treatment with antibiotics fails to cure the pneumonia.

Peripheral carcinoids: These 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 they interfere with breathing. Usually they are found as a spot on a chest x-ray or CT scan taken for an unrelated problem.

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), severe diarrhea, wheezing, and 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, and a heart murmur (an abnormal heart sound).

Cushing syndrome: In rare cases, lung carcinoid tumors may make a hormone called ACTH. This substance causes the adrenal glands to make too much cortisol and other hormones. This can cause weight gain, weakness, high blood sugar (or even diabetes), and 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 right away so the cause can be found and treated, if needed.

Medical history and physical exam: If you have any signs or symptoms that suggest you might have a lung carcinoid tumor, your doctor will want to take a complete medical history, including your family history, to learn about your symptoms and possible risk factors.

A physical exam provides 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 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 may be 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 may be the first imaging test a doctor orders if he or she suspects a lung problem. 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: The CT scan is an x-ray test that produces 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 you while you are lying on a narrow platform. A computer then combines these into images of slices of the part of your body that is 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.

You need to lie still on a table while the scan is being done. During the test, the table slides in and out of the scanner, a ring-shaped machine that completely surrounds it. You might feel a bit confined by the ring you have to lie in while the pictures are being taken.

CT scans can have several uses:

CT scans of the chest can spot very small lung tumors and help determine the exact location and extent of the tumors.

CT scans can be helpful in staging a cancer (determining the extent of its spread). For example, CT scans of the abdomen can show if the cancer has spread to the liver or other organs. This can help to determine if surgery is a good treatment option.

CT scans can also be used to guide a biopsy needle precisely into a suspected tumor or metastasis. For this procedure, called a CT-guided needle biopsy, the patient remains on the CT scanning table, while a radiologist 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.

CT scans can be done during or after treatment to see how effective the 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 determine the extent of the tumor, as well as help locate it if doctors aren't sure where it is in the body.

Somatostatin receptor scintigraphy: The most commonly used 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. About 4 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.

I-131 MIBG: This test is used less often. It uses a chemical called MIBG to which radioactive iodine (I-131) is attached. 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): A PET scan is another imaging test that uses low levels of radioactivity to look for tumors. For most diseases, PET scans use a form of radiolabeled glucose (sugar) to find tumors. This type of PET scan is not very useful in finding carcinoid tumors. 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 finds a mass, it is 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 more likely to help find cancers that start in the major airways of the lung. It may not be as helpful for finding cancers in other parts of the lungs. Sputum cytology 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.

Bronchoscopic biopsy: This approach is used to view and sample tumors of large airways, such as central carcinoids. The doctor passes a long, thin, flexible, fiber-optic tube called a bronchoscope down the throat, through the windpipe, and into the lungs to look at the lining of the lung's main airways. You will be sedated for this. If a tumor is found, the doctor can take a small sample 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 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 surgical incision 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 of a sample 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 a serious problem. 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): EBUS uses a special bronchoscope that has a small balloon on the end. This special bronchoscope is inserted like a regular bronchoscope. The balloon gives off sound waves and collects information about the waves that bounce back (like an ultrasound transducer), showing lymph nodes around the trachea (windpipe).

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 minimally invasive approach can be used to biopsy these nodes. Under ultrasound guidance, a needle can be inserted through the wall of the bronchus into these lymph nodes to remove some cells, which are then looked at under the microscope.

Needle biopsies: Tumors that are toward the outside of the lungs (and not near the large airways) are often sampled by needle biopsy. A long, hollow needle is passed through the skin in the chest between the ribs and into the lung. CT scan images are used to guide the needle into the tumor so that a small sample can be removed and looked at under the microscope. This procedure is done without a surgical incision or overnight hospital stay.

A possible complication of this approach is the buildup of air between the lung and the chest wall, which is known as a pneumothorax. This often goes away on its own, but sometimes it can lead to the collapse of part of a lung, causing shortness of breath. If this happens, it can be treated by temporarily placing a suction tube through the skin and into the chest, which will re-expand the lung.

Surgical biopsies: In some cases, neither a bronchoscopic biopsy nor a needle biopsy will 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.

Thoracotomy: For a thoracotomy, the surgeon makes an incision 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 is less invasive than a thoracotomy. It is most often done in the operating room while you are under general anesthesia (in a deep sleep).

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 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 whether 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 between the lungs. It is done in an operating room while you are under general anesthesia (in a deep sleep). A small cut is made in the front of the neck above the breastbone (sternum) and a thin, hollow, lighted tube is inserted behind the sternum. Special instruments can be passed through this tube to take tissue samples from the lymph nodes along the windpipe and the major bronchial tube areas.

Blood and urine tests: Because carcinoid tumors can secrete hormone-like chemicals into the blood, the tumor can sometimes be detected by simple blood or urine tests. This is especially true if you have symptoms of the carcinoid syndrome, which is caused by excessive levels of these substances in the blood.

Serotonin is a substance 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 commonly used to look for carcinoids can 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 is an option in treating the cancer. Because surgery will remove part or all of the lung, it's important to know how well the lungs are working beforehand. These tests can give the surgeon an idea of whether surgery is a good option, and if so, how much lung can safely be removed.

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.

How are lung carcinoid tumors staged?

Staging is a process of finding out how far a cancer 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, biopsies, and imaging tests (CT scan, PET scan, etc.),

The staging system for lung carcinoid tumors is the same one 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 how much the cancer has spread 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 letter X means cannot be assessed because the information is not available.

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 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.

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. If the tumor is 5 cm or less across (but still larger than 3 cm), it is called T2a. If the tumor is larger than 5 cm across (but not larger than 7 cm), it is called T2b.

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 develop pneumonia.

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.

N categories

NX: Nearby lymph nodes cannot be assessed.

N0: There is no 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 it has spread 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: No spread to distant organs or areas. This includes other lobes of the lungs, lymph nodes further away than those mentioned in the N stages above, and other organs or tissues such as the liver, bones, or brain.

M1: The cancer has spread to 1 or more distant sites. This can be to another lobe of the lung, to distant lymph nodes, or to other organs.

M1a: Any of the following:

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 organs or lymph nodes.

Stage grouping for lung cancer and lung carcinoid: Once the T, N, and M categories have been determined, this information is combined (stage grouping) to assign an overall stage of 0, I, II, III, or IV. Some stages are subdivided into A and B. The stages identify tumors that have a similar prognosis and so they are treated in a similar way. Patients with lower stage numbers tend to have a better prognosis.

Occult cancer: TX, N0, M0: Cancer cells are seen in a sample of sputum or other lung fluids, but the location of the cancer 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 between 3 and 5 cm across (larger than 3 cm but not larger than 5 cm).

The tumor involves a main bronchus, but is not within 2 cm of the carina.

The tumor has grown into the visceral pleura (the membranes surrounding the lungs).

The cancer is partially clogging the airways.

The cancer has not spread to lymph nodes or distant sites.

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 between 3 and 5 cm across (larger than 3 cm but not larger than 5 cm).

The tumor involves a main bronchus, but is not within 2 cm of the carina.

The tumor has grown into the visceral pleura (the membranes surrounding the lungs).

The cancer is partially clogging the airways.

It 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 between 5 and 7 cm across (larger than 5 cm but not larger than 7 cm).

The tumor involves a main bronchus, but is not within 2 cm of the carina.

The tumor has grown into the visceral pleura (the membranes surrounding the lungs).

The cancer is partially clogging the airways.

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:

The main tumor is between 5 and 7 cm across (larger than 5 cm but not larger than 7 cm).

The tumor is larger than 5 cm across and involves a main bronchus, but is not within 2 cm of the carina.

The tumor is larger than 5 cm across and has grown into the visceral pleura (the membranes surrounding the lungs).

The tumor is larger than 5 cm across and is partially clogging the airways.

It 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 involves a main bronchus without growing into the carina.

The tumor has grown into the visceral pleura (the membranes surrounding the lungs).

The cancer is partially clogging the airways.

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 two lungs (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 two 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 does not involve 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.

It 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.

It may also 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: 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:

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).                                         OR

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 another organ.

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