Nasopharyngeal carcinoma is cancer that occurs in the nasopharynx, which is located behind your nose and above the back of your throat.

Nasopharyngeal carcinoma is rare in the United States. In other parts of the world — specifically Asia and northern Africa — nasopharyngeal carcinoma occurs much more frequently.

Nasopharyngeal carcinoma is difficult to detect early, probably because the nasopharnyx isn't easy to examine and symptoms of nasopharyngeal carcinoma mimic those of many other conditions. Your main treatment options for nasopharyngeal carcinoma are radiation therapy, chemotherapy or a combination of the two. You can work with your doctor to determine the exact approach depending on your particular situation.

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Nasopharynx

In its early stages, nasopharyngeal carcinoma may not cause any symptoms. Possible noticeable symptoms of nasopharyngeal carcinoma include:

• A lump in your neck caused by a swollen lymph node
• Bloody discharge from your nose
• Nasal congestion on one side of your nose
• Hearing loss in one ear
• Frequent ear infections
• Headaches
• Double vision
• Face and neck pain

When to see a doctor
Early nasopharyngeal carcinoma symptoms may not always prompt you to see your doctor. However, if you notice any unusual and persistent changes in your body, such as unusual nasal congestion, that don't seem right to you, see your doctor.

Cancer begins when one or more genetic mutations cause normal cells to grow out of control, invade surrounding structures and eventually spread (metastasize) to other parts of the body. In nasopharyngeal carcinomas, this process begins in the squamous cells that line the surface of the nasopharynx.

Exactly what causes the gene mutations that lead to nasopharyngeal carcinoma isn't known, though risk factors, such as the Epstein-Barr virus, that increase the risk of this cancer have been identified. However, it isn't clear why some people with all the risk factors never develop cancer, while others who have no apparent risk factors do.

Researchers have identified some risk factors that appear to increase your risk of developing nasopharyngeal carcinoma, including:

• Sex. Men have about double the risk of developing cancer of the nasopharnyx as women do.
• Race. This type of cancer more commonly affects people in Asia and northern Africa. In the United States, Asian immigrants have a higher risk of this type of cancer than do American-born Asians, which may be related to differences in diet. The Inuits of Alaska also have an increased risk of nasopharyngeal cancer. Additionally, blacks are significantly more likely to develop nasopharyngeal cancer than are whites.
• Age. Most cases of nasopharyngeal cancer occur in people between the ages of 30 and 55.
• Salt-cured foods. Chemicals released in steam when cooking salt-cured foods, such as fish, preserved vegetables and Chinese herbs, may enter the nasal cavity, increasing the risk of nasopharyngeal carcinoma. In China, nasopharyngeal carcinoma has been linked to high consumption of salted fish, and as people in Southeast China are adopting a more Western diet, their rates of nasopharyngeal cancer have been declining.
• Preserved meats. Preserved meats contain high levels of nitrates, which may increase the risk of nasopharyngeal carcinoma.
• Epstein-Barr virus. This common virus usually produces mild signs and symptoms, such as those of a cold. Sometimes it can cause infectious mononucleosis. Epstein-Barr virus is also linked to several rare cancers, including nasopharyngeal carcinoma. In fact, the Epstein-Barr virus can be found in almost all nasopharyngeal cancer cells.
• Family history. Having a family member with nasopharyngeal carcinoma increases your risk of the disease, though researchers aren't sure if this association is due to genetic or environmental factors.

Nasopharyngeal carcinoma frequently spreads (metastasizes) beyond the nasopharynx. Most people with nasopharyngeal carcinoma have regional metastases, meaning cancer cells from the initial tumor have migrated to nearby areas, such as lymph nodes in the neck. Cancer cells that spread beyond the head and neck (distant metastases) most commonly travel to the bones and bone marrow, lungs and liver.

Nasopharyngeal carcinoma may also cause paraneoplastic syndromes. In these rare disorders, the cancer produces substances that cause changes in other parts of your body. The changes aren't a direct effect of cancer cells, however. Paraneoplastic syndromes may cause high levels of certain white blood cells in your blood, neurological problems or joint problems. Once your cancer is treated, your doctor may prescribe medications to control your immune system.

If your doctor suspects or has diagnosed nasopharyngeal cancer, you may be referred to a doctor who specializes in treating cancer (oncologist) or to a doctor who specializes in ear, nose and throat problems (otolaryngologist).

Because appointments can be brief, and it can be difficult to remember everything you wanted to discuss, it's a good idea to be well prepared for your appointment.  Here are some suggestions for preparing for your appointment, and what you can expect from your doctor.

What you can do

• Write down any symptoms you're experiencing. Include all of your symptoms, even if you don't think they're related.
• Make a list of any medications or vitamin supplements you take. Write down doses and how often you take them.
• Take a family member or close friend with you, if possible. You may be given a lot of information at your visit, and it can be difficult to remember everything. If someone else is with you, he or she may remember details that you missed or forgot. He or she may also think to ask important questions you might not have considered.
• Take a notebook or note pad with you. That way you can write down important information, such as treatment options.
• Prepare a list of questions to ask your doctor. Knowing ahead of time what you want to ask your doctor can help you make the most of your limited time together.

List your most important questions first, in case time runs out. For nasopharyngeal carcinoma, some basic questions to ask include:

• What kinds of tests will I need?
• Do I need to do anything to prepare for these tests?
• Other than nasopharyngeal cancer, are there any possible causes for these symptoms?
• What type of nasopharyngeal cancer do I have?
• Has the cancer spread beyond the nasopharynx? Beyond the lymph nodes?
• What stage is my cancer?
• What is the usual treatment for this stage of cancer?
• Do you recommend that radiation and chemotherapy be done at the same time?
• How successful is each treatment on its own, and when combined?
• What are the side effects of radiation?
• What are the side effects of chemotherapy?
• Is surgery an option?
• How should I prepare for treatment?
• Which course of action do you recommend?
• What are the odds of recurrence?
• Should I modify my diet in any way?
• What is my prognosis?
• Are any clinical trials available to me?

And, if your doctor says something that's not clear, don't hesitate to ask him or her questions until you understand completely.

What to expect from your doctor
Your doctor will likely have a number of questions for you. If you're ready to answer them, it may save time for the rest of your appointment. Some potential questions your doctor might ask include:

• When did you first notice these symptoms?
• How often do you experience these symptoms?
• How severe are your symptoms?
• Does anything improve your symptoms?
• Does anything make your symptoms worse?
• Tell me about your diet.
• Have you ever been diagnosed with Epstein-Barr?

Screening
In the United States and in other areas where the disease is rare, routine screening for nasopharyngeal carcinoma isn't done. But in areas of the world where nasopharyngeal carcinoma is much more common, for instance in some areas of China, doctors may offer screenings to people thought to be at high risk of the disease. Screenings may include blood tests to determine whether a person carries the Epstein-Barr virus and what the levels of the virus are, or careful examinations of the nasopharynx using a tiny camera attached to the end of a flexible tube (endoscope).

Diagnosis
Diagnosing nasopharyngeal carcinoma usually begins with a general examination. Your doctor will ask questions about your symptoms. He or she may press on your neck to feel for swelling in your lymph nodes. Because early signs and symptoms of nasopharyngeal carcinoma aren't specific to the disease, the initial diagnosis is difficult to make. It may take months of investigating other avenues before a definitive diagnosis is made.

If nasopharyngeal carcinoma is suspected, your doctor will refer you to an ear, nose and throat specialist (otolaryngologist) who will use an endoscope to see inside your nasopharynx and look for abnormalities. The endoscope may be inserted through your nose or through the opening in the back of your throat that leads up into your nasopharynx. Endoscopy may require local anesthesia.

Your doctor may also use the endoscope or another instrument to take a small tissue sample (biopsy) to be tested for cancer. Beyond diagnosing nasopharyngeal cancer, a biopsy also tells your doctor the type of nasopharyngeal carcinoma you have.

Nasopharyngeal carcinoma is divided into three types based on the appearance of the cells when viewed under a microscope:

• Type 1 is called keratinizing squamous cell carcinoma.
• Type 2 is nonkeratinizing carcinoma.
• Type 3 is called undifferentiated carcinoma.

Which type you have may depend on where you live. Type 1 is the most common type in the United States, while type 3 is the most common type seen in Southeast Asia. Your doctor may factor in your specific type of nasopharyngeal carcinoma when selecting your treatment, though treatments are generally the same for each type.

Staging
Once the diagnosis is confirmed, your doctor orders other tests to determine the extent (stage) of the cancer, such as:

• Computerized tomography (CT). CT scans show whether the cancer has expanded into the surrounding bone. CT scans capture X-ray images, but rather than a couple of views of a certain area, CT scanners capture multiple, small "slices" of your body, giving your doctor a much more detailed view.

  Generally, no special preparation is needed for a CT scan, though you may need to have a contrast medium injected through an intravenous line as part of the test. The contrast medium further highlights your body's structures. Rarely, the contrast medium may cause an allergic reaction, so let your doctor know if you've ever experienced an allergic reaction to a contrast medium in the past, and let the doctor know if you have kidney problems, as the dye can adversely affect the kidneys.

  For the test, you lie on a table that pulls you into the doughnut-shaped CT scanner. As with an X-ray, you have to lie still while the images are being captured. CT scans usually don't take too long, and some newer machines take only seconds to perform the test.

• Magnetic resonance imaging (MRI). MRI helps show whether the cancer has expanded to nearby soft tissues in your head and neck. This test uses magnets and radio waves to transmit images to a nearby computer. As with CT scans, special preparation usually isn't necessary, but a contrast medium may be used to provide additional detail. MRI scans take longer than X-ray or CT scans do, possibly up to one hour.

  Some people are uncomfortable in MRI machines because the space is confined and the machine makes a loud thumping noise during the test. Many MRI centers provide music via headphones to help block out some of the noise. If you're afraid of small spaces or of being confined, talk with your doctor before the test. A mild sedative might make the test more comfortable for you.

• Chest X-ray or CT scan. X-ray or CT scan of the chest may show whether cancer has spread to your lungs.
• Positron emission tomography (PET) scan. PET scans use glucose combined with a radioactive atom to highlight areas where cancer occurs. PET scanners can detect and map the radioactive atoms, which are found more often in cancerous cells because cancer cells use more energy, and thus more glucose, than healthy cells do. This test can be especially useful if your doctor thinks the cancer has spread to distant areas. Some medical centers have combined CT/PET scanners.
• Lymph node biopsy. Doctors check the lymph nodes in your neck (cervical nodes) for signs of cancer by performing a biopsy. In some cases you may undergo surgery to remove an entire lymph node through a small incision in the skin. In other cases a biopsy may be performed using a procedure called fine-needle aspiration, in which your doctor inserts a needle into the lymph nodes to search for cancer cells.

Once your doctor has determined the extent of your cancer, he or she assigns it a stage. The stage is used along with several other factors to determine your treatment plan and your prognosis. The stages of nasopharyngeal carcinoma include:

• Stage 0. The cancer is limited to the lining of the nasopharynx. This is also called nasopharyngeal carcinoma in situ.
• Stage I. Cancer is confined to the nasopharynx.
• Stage II. Cancer has spread beyond the nasopharynx either to your nasal cavity or to the soft tissues of your throat, including the soft palate, the base of your tongue or your tonsils. In stage IIA, the cancer hasn't spread to your lymph nodes. In stage IIB, the cancer has spread to one or more of the lymph nodes on the same side of your neck as the original tumor, but hasn't spread to distant sites.
• Stage III. Cancer has spread to the lymph nodes on both sides of your neck and may or may not have spread to the soft tissues of your throat. Or, cancer has spread to your throat and the lymph nodes on one or both sides of your neck. Or, cancer has spread to nearby bones and the lymph nodes on one or both sides of your neck.
• Stage IV. Cancer has spread to either the nerves in your face, the lower portion of your throat, the bones of your skull or the bones around your eyes. Or cancer has spread to the lymph nodes in your neck, causing them to grow larger than about 2 1/2 inches (about 6 centimeters). Or, cancer has spread to other parts of your body.

Staging in various parts of the world may be done on a different scale. For instance, a staging system used in Asia includes a stage V.

You and your doctor work together to devise a treatment plan based on several factors, such as the stage of your cancer, the type of cells involved, your treatment goals, your overall health and the side effects you're willing to tolerate.

Many people aren't diagnosed with nasopharyngeal carcinoma until it has spread, probably because the nasopharnyx isn't easy to examine and symptoms of this cancer mimic those of many other conditions.  As nasopharyngeal carcinoma becomes more advanced, it also becomes more difficult to treat successfully.

Treatment for nasopharyngeal carcinoma usually begins with radiation therapy, or a combination of radiation and chemotherapy. While surgery is the mainstay of treatment for many cancers, navigating the nasopharynx with surgical tools is delicate. Nasopharyngeal carcinoma is particularly sensitive to radiation therapy, making it or combined chemoradiotherapy the first line of treatment.

Radiation
Radiation therapy destroys quickly growing cells, including cancer cells, in the area where the beams are focused. During treatment you're positioned on a table and a large machine is maneuvered around you to the precise spot where it can target your cancer. You typically receive radiation treatment five days a week for six or seven weeks. Some doctors are using a newer form of radiation called intensity-modulated radiation therapy (IMRT). This type of radiation is more effective at contouring to the shape of the tumor, so it may affect fewer healthy cells.

Radiation therapy carries a risk of side effects, including temporary skin changes, hearing loss, dry mouth and difficulty swallowing. Your nasopharynx is situated among some delicate organs, such as your brain, spinal cord, thyroid gland, eyes and ears. Your radiation therapy team works to protect these organs, but that can't always be done.

Internal radiation therapy (brachytherapy) is sometimes used in recurrent nasopharyngeal carcinoma.  With this treatment, radioactive seeds or wires are positioned in the tumor or very close to it.

Chemotherapy
Unlike radiation therapy, which is focused on one part of your body, chemotherapy medications travel throughout your body. Chemotherapy works by attacking quickly growing cells, including cancer cells. Some healthy cells are also killed by chemotherapy, which can cause side effects, including fatigue, hair loss, and nausea and vomiting. Chemotherapy may be used to treat nasopharyngeal carcinoma in three ways:

• Chemotherapy at the same time as radiation therapy. This is called concomitant therapy or chemoradiotherapy. Chemotherapy enhances the effectiveness of radiation therapy. Using the two treatments together may reduce the need for high doses of radiation, which can reduce the side effects associated with radiation therapy. However, side effects of chemotherapy are added to the side effects of radiation therapy, making concomitant therapy more difficult to tolerate.
• Chemotherapy after radiation therapy. This is called adjuvant chemotherapy. Your doctor might recommend adjuvant chemotherapy after radiation therapy alone or after concomitant therapy. Adjuvant chemotherapy is used to attack any remaining cancer cells in your body, including those that may have broken off from the original tumor and spread elsewhere. Some controversy exists as to whether adjuvant chemotherapy actually improves survival in people with nasopharyngeal carcinoma. Many people who undergo adjuvant therapy after concomitant therapy are unable to tolerate the side effects and must discontinue treatment.
• Chemotherapy before radiation therapy. This is called neoadjuvant chemotherapy. It works in the same way as adjuvant chemotherapy, but it's administered before radiation therapy alone or before concomitant therapy. However, little research has been done on neoadjuvant chemotherapy, so it's not considered a standard treatment.

What chemotherapy drugs you receive and how often will be determined by your doctor. The side effects you're likely to experience will depend on which drugs you receive.

Surgery
Surgery is not often used as a treatment for nasopharyngeal carcinoma. Surgery to remove cancerous lymph nodes in the neck is the most common surgery for nasopharyngeal carcinoma. Surgery to remove a tumor from the nasopharynx requires surgeons to make an incision in the roof of your mouth in order to access the area to remove the cancerous tissue.

Everyone deals with a cancer diagnosis in his or her own way. You might experience shock and fear after your diagnosis. Allow yourself time to grieve. A cancer diagnosis can make you feel as though you have little control, so take steps to empower yourself and control what you can about your health. Try to:

• Learn everything you can about your cancer. Write down all the questions that are racing through your mind and ask them at the next appointment with your doctor. Get a friend or family member to come to appointments with you to take notes. Ask your health care team for further sources of information. The more you know about your condition, the better prepared you'll be to make decisions about your treatment. Contact the National Cancer Institute for information online or by telephone at 800-4-CANCER (800-422-6237). The American Cancer Society also offers support and information on its Web site and by telephone at 800-ACS-2345 (800-227-2345).
• Create a support network. Having friends and family around you and supporting you can be valuable. You may find it helps to have someone to talk to about your emotions. Other people who may provide support include social workers and psychologists — ask your doctor for a referral if you feel like you need someone to talk to. Talk with your pastor, rabbi or other spiritual leader. Other people with cancer can offer a unique perspective, so consider joining a support group — whether it's in your community or online. Contact the American Cancer Society for more information on support groups.
• Take time for yourself when you need it. Let people know when you want to be alone. Quiet time to think or write in a journal can help you sort out all the emotions you're feeling.
• Take care of yourself. Prepare yourself for treatment by making healthy lifestyle choices. For instance, if you smoke, quit smoking. Eat a variety of fruits and vegetables, and restrict the amount of salt-cured foods you eat, as these have been linked to the initial development of nasopharyngeal carcinoma. Get some exercise when you feel up to it, but check with your doctor before starting a new exercise program. Try to get enough sleep so that you wake feeling refreshed. Talk to your doctor if you're having trouble sleeping. Try to control stress by prioritizing what's important to you. These healthy choices make it easier for your body to cope with the side effects of treatment.

No sure way exists to prevent nasopharyngeal carcinoma. However, you can take steps to reduce your risk of the disease. For instance, cut back on the amount of salt-cured foods and preserved meats that you eat, or avoid these foods altogether. In parts of China where people have begun to adopt a typical American diet, the incidence of nasopharyngeal carcinoma has been declining.

Although there's no vaccine available to prevent nasopharyngeal cancer, researchers are hopeful that one day they may be able to develop a vaccine for the Epstein-Barr virus, which would likely reduce the risk of nasopharyngeal carcinoma as well.