Childhood Nasopharyngeal Cancer Treatment

Nasopharyngeal cancer is a disease in which malignant (cancer) cells form in the tissues of the nasal cavity and throat.

Nasopharyngeal cancer is a disease in which malignant (cancer) cells form in the nasopharynx. The nasopharynx is made of the nasal cavity (inside of the nose) and top part of the throat.

Nasopharyngeal cancer is more common in teens than in children younger than 10 years of age.

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Anatomy of the nasopharynx. The nasopharynx is in the upper part of the pharynx (throat) behind the nose. The nostrils lead into the nasopharynx. An opening on each side of the nasopharynx leads into the ear.

Being infected with the Epstein-Barr virus increases the risk of nasopharyngeal cancer.

Anything that increases your chance 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 child's doctor if you think your child may be at risk.

Being infected with the Epstein-Barr virus (EBV) is strongly linked to having nasopharyngeal cancer.

Signs and symptoms of nasopharyngeal cancer include headache and blocked or stuffy nose.

These and other signs and symptoms may be caused by nasopharyngeal cancer or by other conditions.

Check with your child's doctor if your child has any of the following:

• Headache.
• Blocked or stuffy nose.
• Nosebleeds.
• Earache.
• Ear infection.
• Hearing loss.
• Problems moving the jaw.
• Trouble speaking.
• Trouble seeing or droopy eyelid.
• Lumps in the neck that may be painful.

Tests that examine the nasopharynx are used to help diagnose nasopharyngeal cancer.

The following tests and procedures may be used:

• Physical exam and health history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
• MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas of the body, such as the head and neck. This procedure is also called nuclear magnetic resonance imaging (NMRI).
• Nasal endoscopy: A procedure to look at organs and tissues inside the body to check for abnormal areas. A flexible or rigid endoscope is inserted through the nose. 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, which are checked under a microscope by a pathologist for signs of disease.
• Epstein-Barr virus (EBV) tests: Blood tests to check for antibodies to the Epstein-Barr virus and DNA markers of the Epstein-Barr virus. These are found in the blood of patients who have been infected with EBV.

Certain factors affect prognosis (chance of recovery).

Prognosis depends on:

• Whether the cancer has just been diagnosed or has recurred (come back).

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

To plan treatment, it is important to know whether cancer cells have spread within the nasal cavity or to other parts of the body. The process used to find out if cancer has spread is called staging.

Most children with nasopharyngeal cancer are at an advanced stage at the time of diagnosis. Nasopharyngeal cancer spreads most often to the bone, lung, and liver.

The following tests and procedures may be used to find out if cancer has spread:

• 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.
• Chest x-ray: An x-ray of the organs and bones inside the chest. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
• PET-CT scan: A procedure that combines the pictures from a PET scan and a CT scan. The PET and CT scans are done at the same time on the same machine. The pictures from both scans are combined to make a more detailed picture than either test would make by itself.
  • CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest or abdomen, 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.
  • 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.
• Bone scan: A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones with cancer and is detected by a scanner.
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  Bone scan. A small amount of radioactive material is injected into the child's vein and travels through the blood. The radioactive material collects in the bones. As the child lies on a table that slides under the scanner, the radioactive material is detected and images are made on a computer screen.
  

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.

Sometimes childhood nasopharyngeal cancer does not respond to treatment or comes back after treatment.

Refractory nasopharyngeal cancer is cancer that does not respond to treatment.

Recurrent nasopharyngeal cancer is cancer that has recurred (come back) in the nasopharynx or in other parts of the body such as the bone, lung, or liver.

There are different types of treatment for children 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.

Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.

Children with nasopharyngeal cancer should have their treatment planned by a team of doctors who are experts in treating childhood cancer.

Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health professionals who are experts in treating children with cancer and who specialize in certain areas of medicine. This may include the following specialists and others:

• Pediatrician.
• Pediatric surgeon.
• Radiation oncologist.
• Pediatric ear, nose, and throat specialist.
• Pathologist.
• Pediatric nurse specialist.
• Social worker.
• Rehabilitation specialist.
• Psychologist.
• Child-life specialist.

Four types of standard treatment are used:

Immunotherapy

Immunotherapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This cancer treatment is a type of biologic therapy.

Several types of immunotherapy are being used or studied to treat children with nasopharyngeal cancer:

• Interferon may stop cancer cells from growing and it may also help kill cancer cells.
• EBV-specific cytotoxic T-lymphocytes are a type of immune cell that can kill certain cells, including foreign cells, cancer cells, and cells infected with the Epstein-Barr virus. Cytotoxic T-lymphocytes can be separated from other blood cells, grown in the laboratory, and then given to the patient to kill cancer cells. EBV-specific cytotoxic T-lymphocytes are being studied to treat refractory or recurrent nasopharyngeal cancer.
• Immune checkpoint inhibitor therapy blocks certain proteins. PD-1 is a protein on the surface of T cells that helps keep the body’s immune responses in check. PD-L1 is a protein found on some types of cancer cells. When PD-1 attaches to PD-L1, it stops the T cell from killing the cancer cell. PD-1 and PD-L1 inhibitors keep PD-1 and PD-L1 proteins from attaching to each other. This allows the T cells to kill cancer cells. Pembrolizumab and nivolumab are types of PD-1 inhibitors that have been used to treat adults with refractory nasopharyngeal cancer and may be considered to treat children.

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Immune checkpoint inhibitor. Checkpoint proteins, such as PD-L1 on tumor cells and PD-1 on T cells, help keep immune responses in check. The binding of PD-L1 to PD-1 keeps T cells from killing tumor cells in the body (left panel). Blocking the binding of PD-L1 to PD-1 with an immune checkpoint inhibitor (anti-PD-L1 or anti-PD-1) allows the T cells to kill tumor cells (right panel).

New types of treatment are being tested in clinical trials.

Information about clinical trials is available from the NCI website.

Treatment for childhood nasopharyngeal cancer may cause side effects.

To learn more about side effects that begin during treatment for cancer, visit Side Effects.

Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include:

• Physical problems that affect the following:
  • Problems with the thyroid gland.
  • Decreased mouth function.
  • Hearing loss or chronic ear infections.
  • Dental cavities.
  • Chronic sinusitis.
• Changes in mood, feelings, thinking, learning, or memory.
• Second cancers (new types of cancer) or other conditions.

Some late effects may be treated or controlled. It is important to talk with your child's doctors about the possible late effects caused by some treatments. See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.

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.

Clinical trials are taking place in many parts of the country. Information about clinical trials supported by NCI can be found on NCI’s clinical trials search webpage. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.

Follow-up tests may be needed.

As your child goes through treatment, they will have follow-up tests or check-ups. Some tests that were done to diagnose or stage the cancer may be repeated 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 child's condition has changed or if the cancer has recurred (come back).

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This PDQ cancer information summary has current information about the treatment of childhood nasopharyngeal cancer. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.

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Clinical Trial Information

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." 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.

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PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Nasopharyngeal Cancer Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/head-and-neck/patient/child/nasopharyngeal-treatment-pdq. Accessed <MM/DD/YYYY>.

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