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Nasopharyngeal carcinoma is cancer that occurs in the nasopharynx, which is located behind the nose and above the back of the throat. The nasopharynx is the upper portion of the pharynx — a 5-inch tube that extends from behind the nose to the top of the windpipe and esophagus in your neck.

Nasopharyngeal carcinoma is rare in the United States, comprising only 2 percent of all head and neck cancers. About 2,000 people are diagnosed with nasopharyngeal carcinoma every year in the United States. In other parts of the world — specifically Southeast Asia and northern Africa — nasopharyngeal carcinoma occurs much more frequently.

Men are more likely than women to develop nasopharyngeal carcinoma. It's most commonly diagnosed in people from age 30 to age 60, though nasopharyngeal carcinoma can also occur in older adults and in children.

Nasopharyngeal carcinoma causes signs and symptoms that may suggest a variety of diseases and conditions. That fact, combined with the hidden location of the nasopharynx, means most people aren't diagnosed with nasopharyngeal carcinoma until the cancer has spread. Unfortunately, as nasopharyngeal carcinoma becomes more advanced, it also becomes more difficult to treat.

Signs and symptoms of nasopharyngeal carcinoma may include:

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

All cancers begin with one or more genetic mutations that cause cells to grow out of control, invade surrounding structures and eventually spread (metastasize) to other parts of the body. Nasopharyngeal carcinomas begin in the squamous cells that line the surface of the nasopharynx.

In many cases, it isn't clear what causes the gene mutations that lead to nasopharyngeal carcinoma, though risk factors 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.

In areas where nasopharyngeal carcinoma is most common, researchers have identified several risk factors, including:

• Salt-cured foods. Chemicals released in steam when cooking salt-cured foods may enter the nasal cavity, increasing the risk of nasopharyngeal carcinoma. In China, nasopharyngeal carcinoma has been linked to high consumption of salted fish.
• 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.
• Family history. Having a family member with nasopharyngeal carcinoma increases your risk of the disease.

Nasopharyngeal carcinoma isn't as closely linked to smoking and excessive alcohol use as most other head and neck cancers are.

Early signs and symptoms of nasopharyngeal carcinoma 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.

Screening
In the United States, routine screening for nasopharyngeal carcinoma isn't done because the disease is rare. But in areas of the world where nasopharyngeal carcinoma is much more common, for instance in southern 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 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 signs and 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, it's common to be misdiagnosed at first. It may take several months of investigating other avenues before a definitive diagnosis is made.

If nasopharyngeal carcinoma is suspected, your doctor 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. Your doctor factors in your type of nasopharyngeal carcinoma when selecting your treatment.

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

• Magnetic resonance imaging (MRI). MRI helps show whether the cancer has expanded to nearby soft tissues in the head and neck.
• Computerized tomography (CT). CT scans show whether the cancer has expanded into the surrounding bone.
• Bone scan. A bone scan is used to determine whether cancer has spread (metastasized) to other bones in your body.
• Chest X-ray or CT scan. X-ray or CT scan of the chest may show whether cancer has metastasized to the lungs.
• 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 biopsy may be performed using a procedure called fine-needle aspiration where 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. Also called nasopharyngeal carcinoma in situ.
• Stage I. Cancer is confined to the nasopharynx.
• Stage II. Cancer may have spread beyond the nasopharynx to the nasal cavity or to the soft tissues of the throat, including the soft palate, the base of the tongue or the tonsils. Or cancer has spread to the lymph nodes on one side of the neck and may or may not have spread to the soft tissues of the throat.
• Stage III. Cancer has spread to the lymph nodes on both sides of the neck and may or may not have spread to the soft tissues of the throat. Or cancer has spread to the throat and the lymph nodes on one or both sides of the neck. Or cancer has spread to nearby bones and the lymph nodes on one or both sides of the neck.
• Stage IV. Cancer may have spread to the nerves in the face, the lower portion of the throat, the bones of the skull or the bones around the eyes. Or cancer has spread to the lymph nodes in the neck, causing them to grow larger than 6 centimeters. Or cancer has spread to other parts of the 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.

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 your body's immune system reacts to the presence of cancer by attacking normal cells. Paraneoplastic syndromes may cause high levels of certain white blood cells in your blood, fever, neurologic problems or joint problems. Once your cancer is treated, your doctor may prescribe medications to control your immune system.

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 and the side effects you're willing to tolerate. Treatment for nasopharyngeal carcinoma usually begins with radiation therapy. 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 the first line of treatment. Surgery and chemotherapy are used in certain cases.

Radiation
Radiation therapy treats cancer with high-energy beams. 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. You'll also receive radiation to your neck, even if there's no evidence your cancer has spread beyond your nasopharynx. This reduces the chance that your cancer will spread and the chance that your cancer will recur.

Radiation therapy carries a risk of side effects, including hearing loss, dry mouth, sores in the mouth and throat, and an increased risk of tongue cancer and bone cancer. 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
Chemotherapy uses drugs to treat cancer. Unlike radiation therapy, which is focused on one part of your body, chemotherapy travels 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 (concomitant therapy). Chemotherapy enhances the effectiveness of radiation therapy. Using the two treatments together may reduce the need for high doses of radiation, which reduces the side effects associated with radiation therapy. Chemotherapy also reduces the risk that your body will become resistant to radiation therapy. However, side effects of chemotherapy are added to the side effects of radiation therapy, making concomitant therapy difficult to tolerate.
• Chemotherapy after radiation therapy (adjuvant therapy). 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 (neoadjuvant therapy). Neoadjuvant chemotherapy works in the same way as adjuvant chemotherapy, but it's administered before radiation therapy alone or before concomitant therapy. Some people find they experience fewer side effects if they undergo chemotherapy before the rest of their treatment. However, little research has been done on neoadjuvant chemotherapy, so it's considered experimental.

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 usually reserved for recurrent 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.

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. Chinese people who immigrate to North America and adopt a typical American diet have a reduced risk of nasopharyngeal carcinoma. However, the risk never completely goes away, which indicates that other unknown or uncontrollable risk factors may play a role in developing nasopharyngeal carcinoma.

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 healthcare 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, or 800-422-6237. The American Cancer Society also offers support and information on its Web site and by telephone at 800-ACS-2345, or 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. 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.