Nasopharyngeal cancer is a disease in which malignant (cancer) cells form in the tissues of the nasopharynx. Ethnic background and being exposed to the Epstein-Barr virus can affect the risk of nasopharyngeal cancer. Signs of nasopharyngeal cancer include trouble breathing, speaking, or hearing. Tests that examine the nose, throat, and nearby organs are used to diagnose and stage nasopharyngeal cancer. Certain factors affect prognosis (chance of recovery) and treatment options.

Throat cancer is a type of head and neck cancer. Throat cancer has different names, depending on which part of the throat is affected. The different parts of your throat are called the oropharynx, the hypopharynx, the nasopharynx, and the larynx, or voice box.

The main risk factors for throat cancer are using tobacco heavy drinking. Certain types of throat cancer also have other risk factors. For example, having HPV is a risk factor for oropharyngeal cancer.

Symptoms of throat cancer may include

• A sore throat that does not go away
• A lump in the neck
• Pain or ringing in the ears
• Trouble swallowing
• Ear pain

To diagnose throat cancers, doctors may do a physical exam and history, imaging tests, and a biopsy. You may also need other tests, depending on the type of cancer. Treatments include surgery, radiation therapy, and chemotherapy. Treatment for some types of throat cancer may also include targeted therapy. Targeted therapy uses substances that attack cancer cells without harming normal cells.

Nasopharyngeal cancer is a disease in which malignant (cancer) cells form in the tissues of the nasopharynx.

The nasopharynx is the upper part of the pharynx (throat) behind the nose. The pharynx is a hollow tube about 5 inches long that starts behind the nose and ends at the top of the trachea (windpipe) and esophagus (the tube that goes from the throat to the stomach). Air and food pass through the pharynx on the way to the trachea or the esophagus. The nostrils lead into the nasopharynx. An opening on each side of the nasopharynx leads into an ear. Nasopharyngeal cancer most commonly starts in the squamous cells that line the nasopharynx.

Anatomy of the pharynx (throat). The pharynx is a hollow tube that starts behind the nose, goes down the neck, and ends at the top of the trachea and esophagus. The three parts of the pharynx are the nasopharynx, oropharynx, and hypopharynx.

Nasopharyngeal cancer is a type of head and neck cancer.

Tests that examine the nose and throat are used to detect (find) and diagnose nasopharyngeal cancer.

The following tests and procedures may be used:

• Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as swollen lymph nodes in the neck or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken.
• Neurological exam : A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a person's mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam.
• Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. The tissue sample is removed during one of the following procedures:
  • Nasoscopy : A procedure to look inside the nose for abnormal areas. A nasoscope is inserted through the nose. A nasoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
  • Upper endoscopy : A procedure to look at the inside of the nose, throat, esophagus, stomach, and duodenum (first part of the small intestine, near the stomach). An endoscope is inserted through the mouth and into the esophagus, stomach, and duodenum. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples. The tissue samples are checked under a microscope for signs of cancer.
• MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.

Computed tomography (CT) scan of the head and neck. The patient lies on a table that slides through the CT scanner, which takes x-ray pictures of the inside of the head and neck.

• PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. PET scans may be used to find nasopharyngeal cancers that have spread to the bone. Sometimes a PET scan and a CT scan are done at the same time. If there is any cancer, this increases the chance that it will be found.
• Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.
• Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:
  • The number of red blood cells, white blood cells, and platelets.
  • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
  • The portion of the blood sample made up of red blood cells.
• Epstein-Barr virus (EBV) test: A blood test to check for antibodies to the Epstein-Barr virus and DNAmarkers of the Epstein-Barr virus. These are found in the blood of patients who have been infected with EBV.
• Hearing test: A procedure to check whether soft and loud sounds and low- and high-pitched sounds can be heard. Each ear is checked separately.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis and treatment options depend on the following:

• The size of the tumor.
• The stage of the cancer, including whether cancer has spread to one or more lymph nodes in the neck.
• A high level of EBV antibodies and EBV-DNA markers in the blood before and after treatment.

Other factors that may affect prognosis include:

• Age.
• Long period of time between biopsy and start of radiation therapy.
• Family history.
• Tobacco smoking.
• Salted fish in the diet.

Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Talk with your doctor if you think you may be at risk. Risk factors for nasopharyngeal cancer include the following:

• Having Chinese or Asian ancestry.
• Being exposed to the Epstein-Barr virus: The Epstein-Barr virus has been associated with certain cancers, including nasopharyngeal cancer and some lymphomas.
• Drinking large amounts of alcohol.

These and other signs and symptoms may be caused by nasopharyngeal cancer or by other conditions. Check with your doctor if you have any of the following:

• A lump in the nose or neck.
• A sore throat.
• Trouble breathing or speaking.
• Nosebleeds.
• Trouble hearing.
• Pain or ringing in the ear.
• Headaches.

Procedures that make pictures of the nose and throat help diagnose nasopharyngeal cancer. The process used to find out if cancer cells have spread to other parts of the body is called staging. Tests and procedures to detect, diagnose, and stage nasopharyngeal cancer are done before planning treatment.

The following tests and procedures may be used:

• Physical exam and history : An exam of the body to check general signs of health, including checking for signs of disease, such as swollen lymph nodes in the neck or anything else that seems unusual. A history of the patient's health habits and past illnesses and treatments will also be taken.
• Neurological exam : A series of questions and tests to check the brain, spinal cord, and nerve function. The exam checks a person's mental status, coordination, and ability to walk normally, and how well the muscles, senses, and reflexes work. This may also be called a neuro exam or a neurologic exam.
• Biopsy : The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. The tissue sample is removed during one of the following procedures:
  • Nasoscopy : A procedure to look inside the nose for abnormal areas. A nasoscope is inserted through the nose. A nasoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
  • Upper endoscopy : A procedure to look at the inside of the nose, throat, esophagus, stomach, and duodenum (first part of the small intestine, near the stomach). An endoscope is inserted through the mouth and into the esophagus, stomach, and duodenum. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples. The tissue samples are checked under a microscope for signs of cancer.
• MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.

Computed tomography (CT) scan of the head and neck. The patient lies on a table that slides through the CT scanner, which takes x-ray pictures of the inside of the head and neck.

• PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do. PET scans may be used to find nasopharyngeal cancers that have spread to the bone. Sometimes a PET scan and a CT scan are done at the same time. If there is any cancer, this increases the chance that it will be found.
• Blood chemistry studies : A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease.
• Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:
  • The number of red blood cells, white blood cells, and platelets.
  • The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
  • The portion of the blood sample made up of red blood cells.
• Epstein-Barr virus (EBV) test: A blood test to check for antibodies to the Epstein-Barr virus and DNAmarkers of the Epstein-Barr virus. These are found in the blood of patients who have been infected with EBV.
• Hearing test: A procedure to check whether soft and loud sounds and low- and high-pitched sounds can be heard. Each ear is checked separately.

KEY POINTS

• After nasopharyngeal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the nasopharynx or to other parts of the body.
• There are three ways that cancer spreads in the body.
• Cancer may spread from where it began to other parts of the body.
• The following stages are used for nasopharyngeal cancer:
  • Stage 0
  • Stage I
  • Stage II
  • Stage III
  • Stage IV
• After surgery, the stage of the cancer may change and more treatment may be needed.
• Nasopharyngeal cancer can recur (come back) after it has been treated.

After nasopharyngeal cancer has been diagnosed, tests are done to find out if cancer cells have spread within the nasopharynx or to other parts of the body.

The process used to find out whether cancer has spread within the nasopharynx or to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment. The results of the tests used to diagnose nasopharyngeal cancer are often also used to stage the disease.

There are three ways that cancer spreads in the body.

Cancer can spread through tissue, the lymph system, and the blood:

• Tissue. The cancer spreads from where it began by growing into nearby areas.
• Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
• Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

• Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
• Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if nasopharyngeal cancer spreads to the lung, the cancer cells in the lung are actually nasopharyngeal cancer cells. The disease is metastatic nasopharyngeal cancer, not lung cancer.

The following stages are used for nasopharyngeal cancer:

Stage 0

In stage 0, abnormal cells are found in the lining of the nasopharynx. These abnormal cells may become cancer and spread into nearby normal tissue. Stage 0 is also called carcinoma in situ.

Stage I

In stage I, cancer has formed and the cancer:

• is found in the nasopharynx only; or
• has spread from the nasopharynx to the oropharynx and/or to the nasal cavity.

Tumor sizes. The size of a tumor may be compared to the size of a pea (1 cm), peanut (2 cm), grape (3 cm), walnut (4 cm), lime (5 cm), egg (6 cm), peach (7 cm), or grapefruit (10 cm).

Stage II

In stage II, one of the following is true:

• Cancer has spread to one or more lymph nodes on one side of the neck and/or to one or more lymph nodes on one or both sides of the back of the throat. The affected lymph nodes are 6 centimeters or smaller. Cancer is found:
  • in the nasopharynx only or has spread from the nasopharynx to the oropharynx and/or to the nasal cavity; or
  • only in the lymph nodes in the neck. The cancer cells in the lymph nodes are infected with Epstein-Barr virus (a virus linked to nasopharyngeal cancer).
• Cancer has spread to the parapharyngeal space and/or nearby muscles. Cancer may have also spread to one or more lymph nodes on one side of the neck and/or to one or more lymph nodes on one or both sides of the back of the throat. The affected lymph nodes are 6 centimeters or smaller.

Stage III

In stage III, one of the following is true:

• Cancer has spread to one or more lymph nodes on both sides of the neck. The affected lymph nodes are 6 centimeters or smaller. Cancer is found:
  • in the nasopharynx only or has spread from the nasopharynx to the oropharynx and/or to the nasal cavity; or
  • only in the lymph nodes in the neck. The cancer cells in the lymph nodes are infected with Epstein-Barr virus (a virus linked to nasopharyngeal cancer).
• Cancer has spread to the parapharyngeal space and/or nearby muscles. Cancer has also spread to one or more lymph nodes on both sides of the neck. The affected lymph nodes are 6 centimeters or smaller.
• Cancer has spread to the bones at the bottom of the skull, the bones in the neck, jaw muscles, and/or the sinuses around the nose and eyes. Cancer may have also spread to one or more lymph nodes on one or both sides of the neck and/or the back of the throat. The affected lymph nodes are 6 centimeters or smaller.

Stage IV

Stage IV is divided into stages IVA and IVB.

• In stage IVA:
  • Cancer has spread to the brain, the cranial nerves, the hypopharynx, the salivary gland in the front of the ear, the bone around the eye, and/or the soft tissues of the jaw. Cancer may have also spread to one or more lymph nodes on one or both sides of the neck and/or the back of the throat. The affected lymph nodes are 6 centimeters or smaller; or
  • Cancer has spread to one or more lymph nodes on one or both sides of the neck. The affected lymph nodes are larger than 6 centimeters and/or are found in the lowest part of the neck.
• In stage IVB: Cancer has spread beyond the lymph nodes in the neck to distant lymph nodes, such as those between the lungs, below the collarbone, or in the armpit or groin, or to other parts of the body, such as the lung, bone, or liver.

After surgery, the stage of the cancer may change and more treatment may be needed.

If the cancer is removed by surgery, a pathologist will examine a sample of the cancer tissue under a microscope. Sometimes, the pathologist’s review results in a change to the stage of the cancer and more treatment after surgery.

Nasopharyngeal cancer can recur (come back) after it has been treated.

The cancer may come back in the nasopharynx or in other parts of the body.

KEY POINTS

• There are different types of treatment for patients with nasopharyngeal cancer.
• Three types of standard treatment are used:
  • Radiation therapy
  • Chemotherapy
  • Surgery
• New types of treatment are being tested in clinical trials.
• Treatment for nasopharyngeal cancer may cause side effects.
• Patients may want to think about taking part in a clinical trial.
• Patients can enter clinical trials before, during, or after starting their cancer treatment.
• Follow-up tests may be needed.

There are different types of treatment for patients with nasopharyngeal cancer.

Different types of treatment are available for patients with nasopharyngeal cancer. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.

Three types of standard treatment are used:

Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy:

• External radiation therapy uses a machine outside the body to send radiation toward the cancer.

External-beam radiation therapy of the head and neck. A machine is used to aim high-energy radiation at the cancer. The machine can rotate around the patient, delivering radiation from many different angles to provide highly conformal treatment. A mesh mask helps keep the patient’s head and neck from moving during treatment. Small ink marks are put on the mask. The ink marks are used to line up the radiation machine in the same position before each treatment.

Certain ways of giving radiation therapy can help keep radiation from damaging nearby healthy tissue. These types of radiation therapy include the following:

• Intensity-modulated radiation therapy (IMRT): IMRT is a type of 3-dimensional (3-D) radiation therapy that uses a computer to make pictures of the size and shape of the tumor. Thin beams of radiation of different intensities (strengths) are aimed at the tumor from many angles. Compared to standard radiation therapy, intensity-modulated radiation therapy may be less likely to cause dry mouth.

• Stereotactic radiation therapy: A rigid head frame is attached to the skull to keep the head still during the radiation treatment. A machine aims radiation directly at the tumor. The total dose of radiation is divided into several smaller doses given over several days. This procedure is also called stereotactic external-beam radiation therapy and stereotaxic radiation therapy.

• Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer.

The way the radiation therapy is given depends on the type and stage of the cancer being treated. External and internal radiation therapy are used to treat nasopharyngeal cancer.

External radiation therapy to the thyroid or the pituitary gland may change the way the thyroid gland works. A blood test to check the thyroid hormone level in the blood is done before and after therapy to make sure the thyroid gland is working properly. It is also important that a dentist check the patient's teeth, gums, and mouth, and fix any existing problems before radiation therapy begins.

Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated.

Chemotherapy may be given after radiation therapy to kill any cancer cells that are left. Treatment given after radiation therapy, to lower the risk that the cancer will come back, is called adjuvant therapy.

See Drugs Approved for Head and Neck Cancer for more information. (Nasopharyngeal cancer is a type of head and neck cancer.)

Surgery

Surgery is a procedure to find out whether cancer is present, to remove cancer from the body, or to repair a body part. Also called an operation. Surgery is sometimes used for nasopharyngeal cancer that does not respond to radiation therapy. If cancer has spread to the lymph nodes, the doctor may remove lymph nodes and other tissues in the neck.

New types of treatment are being tested in clinical trials.

Treatment for nasopharyngeal cancer may cause side effects.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

Treatment of Stage I Nasopharyngeal Cancer

Treatment of stage I nasopharyngeal cancer is usually radiation therapy to the tumor and lymph nodes in the neck.

Treatment of Stage II Nasopharyngeal Cancer

Treatment of stage II nasopharyngeal cancer may include the following:

• Chemotherapy given with radiation therapy, followed by more chemotherapy.
• Radiation therapy to the tumor and lymph nodes in the neck.

Treatment of Stage III Nasopharyngeal Cancer

Treatment of stage III nasopharyngeal cancer may include the following:

• Chemotherapy given with radiation therapy, which may be followed by more chemotherapy.
• Radiation therapy.
• Radiation therapy followed by surgery to remove cancer-containing lymph nodes in the neck that remain or come back after radiation therapy.
• A clinical trial of chemotherapy given before, with, or after radiation therapy.

Treatment of Stage IV Nasopharyngeal Cancer

Treatment of stage IV nasopharyngeal cancer may include the following:

• Chemotherapy given with radiation therapy, followed by more chemotherapy.
• Radiation therapy.
• Radiation therapy followed by surgery to remove cancer-containing lymph nodes in the neck that remain or come back after radiation therapy.
• Chemotherapy for cancer that has metastasized (spread) to other parts of the body.
• A clinical trial of chemotherapy given before, with, or after radiation therapy.

Treatment of Recurrent Nasopharyngeal Cancer

Treatment of recurrent nasopharyngeal cancer may include the following:

• Intensity-modulated radiation therapy, stereotactic radiation therapy, or internal radiation therapy.
• Surgery.
• Chemotherapy.
• A clinical trial of chemotherapy.
• A clinical trial of stereotactic radiation therapy.