Tracheal cancer (cancer of the windpipe)
This information is about a rare cancer called tracheal cancer. The trachea is often called the windpipe.
We hope this information answers your questions. If you have any further questions, you can ask your doctor or nurse at the hospital where you are having your treatment.
The trachea (windpipe) is the tube that connects your mouth and nose to your lungs. It goes on to divide into the two airways (the right bronchus and the left bronchus, together called bronchi), which supply air to each lung.
The trachea is in the neck and lies in front of your oesophagus (gullet), which food passes down. The trachea is about 10-16cm (5-7in) long and is made up of rings of tough, fibrous tissue (cartilage). You can feel these if you touch the front of your neck.
Cancer of the trachea is rare and only makes up about 0.1% (1 in 1,000) of all cancers. The most common types of tracheal cancer are squamous cell carcinoma and adenoid cystic carcinoma. Squamous cell cancers start in the cells that line different parts of the body, such as the airways, the mouth and the gullet. Adenoid cystic cancers are rarer and develop from glandular tissue. They can develop in different parts of the body but more commonly in the head and neck area.
Causes of tracheal cancer
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We don’t know exactly what causes cancer of the trachea. For most people the cause is unknown.
However, smoking is linked with squamous cell cancer of the trachea. This type of tracheal cancer is also more common in men over 60.
There isn’t any evidence linking adenoid cystic carcinoma of the trachea to smoking. Like many cancers, the cause is unknown. However, it seems to affect men and women equally and is more common between the ages of 40 and 60.
Signs and symptoms of tracheal cancer
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The most common symptoms of tracheal cancer are:
a dry cough
a hoarse voice
difficulty in swallowing
fevers, chills and chest infections that keep coming back
coughing up blood
wheezing or noisy breathing.
These symptoms are common in conditions other than cancer. However, it is important to tell your doctor if you have any of these symptoms.
How tracheal cancer is diagnosed
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Your GP will examine you and arrange for any tests that may be necessary. You will be referred to a hospital specialist for these tests and for expert advice and treatment.
The doctor at the hospital will examine you, ask you about your medical history and take blood samples to check your general health.
Cancer of the trachea is rare and can be difficult to diagnose. It may be mistaken for asthma or bronchitis, which sometimes results in a delay in the diagnosis.
You may have some of the following tests to help diagnose your cancer and to find out whether or not the cancer has spread.
The doctor may take some x-rays to begin with, although cancer of the trachea may not always show up on an x-ray.
CT (computerised tomography) scan
A CT scan takes a series of x-rays that build up a three-dimensional picture of the inside of the body. The scan is painless and takes 10-30 minutes. CT scans use a small amount of radiation, which is very unlikely to harm you and won't harm anyone you come into contact with. You will be asked not to eat or drink for at least four hours before the scan.
You may be given a drink or injection of a dye, which allows particular areas in the body to be seen more clearly on the scan. For a few minutes, this may make you feel hot all over. If you’re allergic to iodine or have asthma, you could have a more serious reaction to the injection, so it's important to let your doctor know beforehand.
MRI (magnetic resonance imaging) scan
This test is similar to a CT scan but uses magnetism instead of x-rays to build up a detailed picture of areas of your body. Before the scan, you may be asked to complete and sign a checklist. This is to make sure that it’s safe for you to have an MRI scan.
Before the scan, you’ll be asked to remove any metal belongings including jewellery. Some people are given an injection of dye into a vein in the arm. This is called a contrast medium and can help the images from the scan to show up more clearly. During the test, you'll be asked to lie very still on a couch inside a long cylinder (tube) for about 30 minutes. It is painless but can be slightly uncomfortable, and some people feel a bit claustrophobic during the scan. It’s also noisy, but you’ll be given earplugs or headphones.
A thin, flexible tube is passed down your mouth or nose to examine the trachea. You will be asked not to eat or drink anything for a few hours before it. Just before the test you may be given a mild sedative to help you relax and to relieve any discomfort.
Once you’re comfortable, a local anaesthetic will be sprayed on to the back of your throat, making it numb.
The bronchoscope is then gently passed into your nose or mouth and down into the trachea. The doctor can look through the bronchoscope to check for any abnormalities. Photographs and biopsies can be taken at the same time.
The test may be slightly uncomfortable but only takes a few minutes. You should not eat or drink for at least an hour afterwards, because your throat will be numb.
As soon as the sedation has worn off, you will be able to go home. You shouldn’t drive for 24 hours after the test and should arrange for someone to collect you from the hospital if possible, as you may feel sleepy. You may have a sore throat for a couple of days after your test, but this will soon disappear.
A rigid bronchoscopy is sometimes used to help doctors plan or give treatment. It can help them see the tumour more clearly and keep the trachea steady during the procedure. You will have a general anaesthetic and you may have to stay in hospital overnight.
The results of these tests will help the specialist decide on the best type of treatment for you.
Staging and grading of tracheal cancer
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The stage of a cancer is a term used to describe its size and whether or not it has spread beyond its original site. Knowing the particular type and stage of a cancer helps the doctors decide on the most appropriate treatment for you.
The most commonly used staging system for cancer is called the TNM system:
T refers to the size or position of the primary tumour (where the cancer first started in the body).
N refers to which lymph nodes are affected, if any.
M refers to whether or not the cancer has spread to other parts of the body (metastases).
The T, N and M will often have numbers attached to describe the detail. For example, a T1 tumour may be very small and just in one layer of tissue, whereas a T4 tumour may be larger and spread through several layers of tissue.
The exact details of the T, N and M will depend on the type of cancer.
Number staging system
In addition to TNM staging, you’ll probably hear the doctors use a number staging system. There are usually three or four number stages for each cancer type.
Stage 1 describes a cancer at an early stage when it is usually small in size and hasn’t spread, whereas stage 4 describes cancer at a more advanced stage when it has usually spread to other parts of the body. Stages 2 and 3 are in between these stages.
The number stages are made up of different combinations of the TNM stages. So a stage 1 cancer may be described as either T1, N0, M0 or T2, N0, M0.
Number stages may also be subdivided to give more detailed information about tumour size and spread. For example, a stage 3 cancer may be subdivided into stage 3a, stage 3b and stage 3c. A stage 3b cancer might differ from a stage 3a cancer in either the tumour size or if the cancer has spread to lymph nodes.
Other terms used
You may hear other terms used to describe cancer:
‘Early’ or ‘local’ may be used to describe a cancer that hasn’t spread.
‘Locally advanced’ describes a cancer that has begun to spread into surrounding tissues or nearby lymph nodes.
‘Local recurrence’ means the cancer has come back in the same area after treatment.
‘Secondary’, ‘advanced’, ‘widespread’ or ‘metastatic’ means the cancer has spread to other parts of the body.
Grading refers to the appearance of the cancer cells under the microscope and gives an idea of how the cancer may behave.
Low-grade means that the cancer cells look similar to normal cells. High-grade means the cells look more abnormal. A low-grade tumour will usually grow more slowly and be less likely to spread than a high-grade tumour.
Treatment for tracheal cancer
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Your treatment will depend on a number of factors, including your general health, the position and size of the cancer and whether it has spread anywhere else in the body. The main treatments for cancer of the trachea are surgery and radiotherapy. They can be given alone or in combination.
Chemotherapy is usually given to relieve symptoms. This is known as palliative chemotherapy. Your treatment will usually be carried out in a specialist cancer treatment centre. If you’re having surgery, you will be operated on by a surgeon who specialises in lung and chest surgery.
In early, small cancers an operation may be able to completely remove the tumour. This is specialised surgery and is only carried out in specialist centres. However, in many cases too much of the length of the trachea is affected to remove the cancer and re-join the cut ends of the trachea.
As well as removing the cancer, the surgeon also usually removes some healthy looking tissue surrounding it (known as a clear margin). This tissue is looked at in the laboratory to see if there are any cancer cells there. If it does contain cancer cells, this may mean having another operation to remove more tissue.
After your operation, you may be looked after in a high dependency unit or intensive care for a few days. You will have a wound in your neck where the cut (incision) was made and a drainage tube to remove any extra fluid or blood in the area. Until you can drink properly, you will have fluids given through a drip (infusion). You’ll have regular painkillers to make sure that any discomfort or pain is kept under control. The nurses will help you get up and about as soon as you are well enough. This will help to keep your circulation moving and prevent complications like blood clots.
After surgery, your trachea will be slightly shorter so you will be encouraged not to stretch your head back for a while after your operation. After your surgery, you will be seen regularly by a physiotherapist who will help you do breathing exercises and to cough up any phlegm (sputum). You may cough up some blood-stained sputum for a few days after the operation.
Radiotherapy may be given after surgery to try to reduce the chances of the cancer coming back. It may also be done if there were any cancer cells left behind after the operation.
Radiotherapy uses high energy x-rays to destroy cancer cells, while doing as little harm as possible to normal cells.
It can be used on its own to cure people with early, low-grade cancer of the trachea who are unable to have surgery. Radiotherapy is also given after surgery to reduce the chances of the cancer coming back (adjuvant radiotherapy) or to relieve symptoms (palliative radiotherapy).
Radiotherapy is usually given by aiming the high-energy x-rays at the trachea from a radiotherapy machine. This is known as external beam radiotherapy. You usually have treatment Monday-Friday, with a rest at the weekend. The treatment may be given for 3-7 weeks. The length of time it’s given for depends on the type of tumour you have and its size. Radiotherapy to control symptoms (known as palliative radiotherapy) is usually given over a shorter period of time.
Side effects of radiotherapy
Problems with swallowing
After 2-3 weeks of treatment, the main problem you’re likely to notice is difficulty swallowing. This happens because the radiotherapy can cause inflammation in your gullet (oesophagus). You may also have heartburn and indigestion.
Tell your doctors if you have any of these side effects, as they can give you medicines to help. If you don’t feel like eating or have problems with swallowing, you can replace meals with nutritious, high-calorie drinks. These are available from most chemists and some can be prescribed by your GP.
Radiotherapy can make you feel very tired. Try to get as much rest as you can, especially if you have to travel a long way for treatment.
Some people develop a skin reaction similar to sunburn. Pale skin may become red and sore or itchy. Darker skin may develop a blue or black tinge. You will be given advice on how to look after your skin.
Your hair will fall out within the area of the body where you had radiotherapy, but it usually grows back again after treatment.
Feelings of sickness (nausea)
Your doctor can prescribe anti-sickness (anti-emetic) drugs, which will help relieve nausea.
Most of these side effects should disappear gradually once your treatment is over, but it’s important to tell your doctor if they continue.
Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy the cancer cells. They work by disrupting the growth of cancer cells. Chemotherapy is usually used to help control the cancer or its symptoms (palliative chemotherapy). Chemotherapy drugs that may be used are cisplatin or carboplatin.
Chemotherapy is rarely used for adenoid cystic cancers of the trachea.
The following treatments may be used to relieve symptoms if the tumour is blocking your airway and making it difficult for you to breathe. Some people may have a combination of these treatments. Your specialist will advise you which treatments are appropriate for you.
Internal radiotherapy (brachytherapy)
Internal radiotherapy (brachytherapy) may be used to open up the airway and relieve your symptoms.
A thin tube is put inside your trachea using a bronchoscope. A source of radiation is then put inside this tube close to the tumour. It's left in place for a few minutes to deliver the treatment and is then removed. The treatment doesn't hurt. You usually need only one session of treatment but it can be repeated if needed.
There aren’t usually many side effects from this type of treatment. You may find you have a cough and produce more phlegm (sputum) for a while.
Laser treatment relieves symptoms by burning the tumour with a laser light. It’s carried out under a general anaesthetic. A bronchoscopy| is done while you're asleep, and a flexible fibre is passed through the bronchoscope to aim the laser beam at the tumour. The beam is aimed at the tumour and destroys as much of it as possible. There aren’t usually any side effects and you can go home the following day or the same evening.
This treatment can be repeated, if needed. Sometimes radiotherapy is given as well to try to make the benefits of the treatment last longer.
Cryotherapy uses liquid nitrogen, which is extremely cold, to freeze and destroy cancer cells. It’s carried out under general anaesthetic. Using a bronchoscope, the doctor puts an instrument called a cryoprobe close to the tumour. Liquid nitrogen is then circulated through the probe to kill off parts of the tumour. The doctor moves the probe around until enough of the tumour has been removed to improve your symptoms.
There aren’t usually many side effects with cryotherapy. You may find you cough up more phlegm for a couple of days after the treatment.
Diathermy is also known as electrocautery. This is done through a bronchoscope. The doctor uses a probe heated by an electrical current to destroy cancer cells and relieve symptoms. There aren’t usually many side effects with this treatment.
Photodynamic therapy (PDT)
Photodynamic therapy (PDT) uses laser, or other light sources, combined with a light-sensitive drug to destroy cancer cells. The light-sensitive drug is given as a liquid into a vein. After waiting for the drug to be taken up by the cancer cells, the laser light is directed at the tumour using a bronchoscope. This starts the drug working to destroy the cancer cells.
PDT will make you temporarily sensitive to light and you will need to avoid bright light for between a few weeks and a few months, depending upon the photosensitising drug that is used. Your specialist will tell how long you should avoid bright light for. Other side effects include swelling, inflammation, breathlessness and a cough.
PDT is a relatively new treatment and is only available at some centres. It is not suitable for everyone. Your doctor can give you more information about this.
Sometimes the airway can become narrow due to pressure on it from the outside. This can sometimes be relieved using a small device called a stent, which is put inside the airway to hold it open. The most commonly used stent is a small wire frame. It's inserted through a bronchoscope in a folded up position and as it comes out of the end of the bronchoscope it opens up, like an umbrella. This pushes the walls of the narrowed airway open.
Airway stents are usually put in under a general anaesthetic. When you wake up, you won’t usually feel it and you'll be able to breathe more easily. The stent can stay in your trachea permanently. Side effects from an airway stent can include infection, blockage with mucus and irritation of the airway. This can cause coughing and breathlessness.
Follow-up after treatment for tracheal cancer
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You will have regular check-ups once your treatment has finished. These will often continue for several years, frequently at first and then less often. If you have any problems or notice any symptoms between visits, let your doctor know.
You may have many different emotions, including anger, resentment, guilt, anxiety and fear. These are all normal reactions, and are part of the process many people go through in trying to come to terms with their condition.
Everyone has their own way of coping with difficult situations. Some people find it helpful to talk to family or friends, while others prefer to seek help from people outside their situation. Some people prefer to keep their feelings to themselves. There is no right or wrong way to cope, but help is there if you need it. Our cancer support specialists can give you information about counselling in your area.
This section has been compiled using information from a number of reliable sources, including:
Macchiarini P. Primary tracheal tumours. Lancet Oncology. 2006. 7: 83-91.
Myers, et al. Cancer of the head and neck. 4th edition. 2003. WB Saunders.
UpToDate. www.uptodate.com (accessed September 2012).
Webb, et al. Primary Tracheal malignant neoplasms. Journal of the American College of Surgeons. 2006. 202 (2): 237-246.
With thanks to: Dr D Gilligan, Consultant Clinical Oncologist, who reviewed this edition.
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