Nasopharyngeal Cancer

  • Dana-Farber/Brigham and Women's Cancer Care

    Nasopharyngeal cancer forms in tissues of the nasopharynx, which is the upper part of the throat behind the nose. Most nasopharyngeal cancers are squamous cell carcinomas, and begin in flat cells lining the nasopharynx. Learn about nasopharyngeal cancer and find information on how we support and care for people with nasopharyngeal cancer before, during, and after treatment.

Treatment 

The Head and Neck Oncology Program is dedicated exclusively to treating patients with head and neck cancers, which include cancers of the throat, larynx, nose, sinuses, and mouth.

Our specialists evaluate and treat all types and stages, from early lesions to the rarest and most challenging cases.  We also specialize in the treatment of all forms and stages of salivary gland and thyroid cancer.

As a patient, you will be seen by highly experienced clinicians from numerous specialties, including head and neck surgery, medical and radiation oncology, dentistry, oral surgery, reconstructive surgery, nutrition services, social work, speech, voice, and swallowing therapy.

Beginning with the initial consultation, your team of specialists will work with you to create a comprehensive treatment plan tailored to your type of cancer, as well as your lifestyle and personal needs, to achieve the best possible outcome.

Providers meet regularly to discuss new developments in clinical and basic research. The close relationships between world-class researchers and medical clinicians ensure that the latest research findings are translated into new, effective treatment approaches as quickly as possible.

Learn more about treatment and support for patients with head and neck cancers 

Our clinicians are experts in treating all types of head and neck cancers, including: 

  • Hypopharyngeal cancer
  • Laryngeal cancer
  • Nasopharyngeal cancer
  • Oral cancer
  • Oral cavity cancer
  • Oropharyngeal cancer
  • Paranasal sinus and nasal cavity cancer
  • Pharyngeal cancer
  • Laryngeal cancer
  • Lip cancer
  • Ear and Temporal Bone cancer
  • Skull Base cancer
  • Head and Neck Sarcomas
  • Mandible and jaw cancer
  • Adult craniopharyngioma
  • Unknown primary cancer
  • Merkel cell carcinoma
  • Head and Neck Melanoma
  • Salivary gland cancer
  • Parotid gland cancer
  • Acinic cell cancer
  • Adenoidcystic carcinoma
  • Mucoepidermoid cancer
  • Adenocarcinoma cancer
  • Papillary thyroid cancer
  • Follicular thyroid cancer
  • Anaplastic thyroid cancer
  • Medullary thyroid cancer
  • Hurthle cell cancer

Contact us 

New patients 

If you have never been seen before at Dana-Farber/Brigham and Women’s Cancer Center, please call 877-442-3324 or use this online form to make an appointment.

For all other inquiries, please call 617-632-3090

Referring physicians: 617-632-6869

Fax: 617-632-4448

Mailing address
Head and Neck Oncology Center
Dana-Farber Cancer Institute
450 Brookline Ave.
Boston, MA 02115-5450

Information for: Patients | Healthcare Professionals

General Information About Nasopharyngeal Cancer

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; drawing shows the nasopharynx, oropharynx, and hypopharynx. Also shown are the nasal cavity, oral cavity, esophagus, trachea, and larynx. 
Anatomy of the pharynx (throat). The three parts of the pharynx are the nasopharynx, oropharynx, and hypopharynx.

 

Nasopharyngeal cancer is a type of head and neck cancer.

Ethnic background and being exposed to the Epstein-Barr virus can affect the risk of nasopharyngeal cancer.

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.

Signs of nasopharyngeal cancer include trouble breathing, speaking, or hearing.

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.

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.
  • PET scan (positron emission tomography scan): A procedure to find malignanttumorcells in the body. A small amount of radioactiveglucose (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 in the organ or tissue that makes it.
  • 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 DNA markers 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 (chance of recovery) and treatment options depend on the following:

  • The stage of the cancer (whether it affects part of the nasopharynx, involves the whole nasopharynx, or has spread to other places in the body).
  • The type of nasopharyngeal cancer.
  • The size of the tumor.
  • The patient’s age and general health.

Stages of Nasopharyngeal Cancer

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 diagnosenasopharyngeal cancer are often also used to stage the disease. (See the General Information section.)

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 (Carcinoma in Situ)

In stage 0, abnormalcells 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 nasalcavity.

The oropharynx is the middle part of the throat and includes the soft palate, the base of the tongue, and the tonsils.

Tumor size compared to everyday objects; shows various measurements of a tumor compared to a pea, peanut, walnut, and lime  
Pea, peanut, walnut, and lime show tumor sizes.

Stage II

In stage II nasopharyngeal cancer, the cancer:

  • is found in the nasopharynx only or has spread from the nasopharynx to the oropharynx and/or to the nasalcavity. Cancer has spread to one or more lymph nodes on one side of the neck and/or to lymph nodes behind the pharynx. The affected lymph nodes are 6 centimeters or smaller; or
  • is found in the parapharyngeal space. Cancer may have spread to one or more lymph nodes on one side of the neck and/or to lymph nodes behind the pharynx. The affected lymph nodes are 6 centimeters or smaller.

The oropharynx is the middle part of the throat and includes the soft palate, the base of the tongue, and the tonsils. The parapharyngeal space is a fat-filled, triangular area near the pharynx, between the base of the skull and the lower jaw.

Stage III

In stage III nasopharyngeal cancer, the cancer:

  • is found in the nasopharynx only or has spread from the nasopharynx to the oropharynx and/or to the nasalcavity. Cancer has spread to one or more lymph nodes on both sides of the neck. The affected lymph nodes are 6 centimeters or smaller; or
  • is found in the parapharyngeal space. Cancer has spread to one or more lymph nodes on both sides of the neck. The affected lymph nodes are 6 centimeters or smaller; or
  • has spread to nearby bones or sinuses. Cancer may have spread to one or more lymph nodes on one or both sides of the neck and/or to lymph nodes behind the pharynx. The affected lymph nodes are 6 centimeters or smaller.

The oropharynx is the middle part of the throat and includes the soft palate, the base of the tongue, and the tonsils. The parapharyngeal space is a fat-filled, triangular area near the pharynx, between the base of the skull and the lower jaw.

Stage IV

Stage IV nasopharyngeal cancer is divided into stages IVA, IVB, and IVC.

  • Stage IVA: Cancer has spread beyond the nasopharynx and may have spread to the cranial nerves, the hypopharynx (bottom part of the throat), areas in and around the side of the skull or jawbone, and/or the bone around the eye. Cancer may also have spread to one or more lymph nodes on one or both sides of the neck and/or to lymph nodes behind the pharynx. The affected lymph nodes are 6 centimeters or smaller.
  • Stage IVB: Cancer has spread to lymph nodes between the collarbone and the top of the shoulder and/or the affected lymph nodes are larger than 6 centimeters.
  • Stage IVC: Cancer has spread beyond nearby lymph nodes to other parts of the body.

Recurrent Nasopharyngeal Cancer

Recurrentnasopharyngeal cancer is cancer that has recurred (come back) after it has been treated. The cancer may come back in the nasopharynx or in other parts of the body.

Treatment Option Overview

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. 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 radiation therapy to the thyroid or the pituitary gland may change the way the thyroid gland works. The doctor may test the thyroid gland before and after therapy to make sure it 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.

Intensity-modulated radiation therapy (IMRT) is a type of 3-dimensional 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. This type of radiation therapy causes less damage to healthy tissue near the tumor. Compared to standard radiation therapy, intensity-modulated radiation therapy may be less likely to cause xerostomia (dry mouth). This may improve the patient's quality of life.

Stereotactic radiation therapy uses a rigid head frame attached to the skull to aim radiation directly to a tumor, causing less damage to nearby healthy tissue. 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.

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.

Information about clinical trials is available from the NCI Web site.

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. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.

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. This is sometimes called re-staging.

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 Options by Stage

Stage I Nasopharyngeal Cancer

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

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage I nasopharyngeal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI Web site.

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.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage II nasopharyngeal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI Web site.

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.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage III nasopharyngeal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI Web site.

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.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage IV nasopharyngeal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI Web site.

Treatment Options for Recurrent Nasopharyngeal Cancer

Treatment of recurrentnasopharyngeal 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.

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent nasopharyngeal cancer. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. Talk with your doctor about clinical trials that may be right for you. General information about clinical trials is available from the NCI Web site.

To Learn More About Nasopharyngeal Cancer

For more information from the National Cancer Institute about nasopharyngeal cancer, see the following:

For general cancer information and other resources from the National Cancer Institute, see the following:


This information is provided by the National Cancer Institute.

This information was last updated on December 20, 2013.


General Information About Nasopharyngeal Cancer

Anatomy

The nasopharynx has a cuboidal shape. The lateral walls are formed by the eustachian tube and the fossa of Rosenmuller. The roof, sloping downward from anterior to posterior, is bordered by the pharyngeal hypophysis, pharyngeal tonsil, and pharyngeal bursa with the base of the skull above. Anteriorly, the nasopharynx abuts the posterior choanae and nasal cavity, and the posterior boundary is formed by the muscles of the posterior pharyngeal wall. Inferiorly, the nasopharynx ends at an imaginary horizontal line formed by the upper surface of the soft palate and the posterior pharyngeal wall.

Risk Factors

Unlike other squamous cell cancers of the head and neck, nasopharyngeal cancer does not appear to be linked to excess use of tobacco or moderate alcohol intake (up to 15 drinks a week). Factors thought to predispose to this tumor include the following:

  • Chinese (or Asian) ancestry.[1]
  • Epstein-Barr virus (EBV) exposure.
  • Unknown factors that result in very rare familial clusters.[2]
  • Heavy alcohol intake.[3]

Signs and Symptoms

Symptoms and signs at presentation include the following:

  • Painless, enlarged lymph nodes in the neck (present in approximately 75% of patients and often bilateral and posterior).
  • Nasal obstruction.
  • Epistaxis.
  • Diminished hearing.
  • Tinnitus.
  • Recurrent otitis media.
  • Cranial nerve dysfunction (usually II–VI or IX–XII).
  • Sore throat.
  • Headache.

In the patient who presents with only cervical adenopathy, the finding of EBV genomic material in the tissue after amplification of DNA with the polymerase chain reaction lends strong evidence for a nasopharyngeal primary tumor, and a concerted search should be conducted in that area.[4]

Diagnostic Tests

Diagnosis is made by biopsy of the nasopharyngeal mass. Workup includes the following:[5]

  • Careful visual examination (by fiberoptic endoscopic examination or examination under anesthesia [EUA]).
  • Documentation of the size and location of the tumor and neck nodes.
  • Evaluation of cranial nerve function including neuro-ophthalmological evaluation and audiological evaluation.
  • Computed tomographic (CT) scan or positron emission tomography (PET)-CT scan.
  • Magnetic resonance imaging (MRI) to evaluate skull base invasion.
  • Hemogram.
  • Chemistry panel.
  • Epstein-Barr virus titers.

Any clinical or laboratory suggestion of distant metastasis may prompt further evaluation of other sites. Careful dental and oral hygiene evaluation and therapy is particularly important prior to initiation of radiation treatment. MRI is often more helpful than CT scans in assessing skull base involvement and in defining the extent of abnormalities detected.[5][6][7]

Prognosis

Major prognostic factors adversely influencing outcome of treatment include the following:[8]

  • Large tumor size.[9][Level of evidence: 3iiiA]
  • A higher tumor (T) stage.
  • The presence of involved neck nodes.

Other factors linked to diminished survival that were present in some, but not all, studies include the following:

  • Age.
  • World Health Organization (WHO) grade I.
  • Long interval between biopsy and initiation of radiation therapy.
  • Diminished immune function at diagnosis.
  • Incomplete excision of involved neck nodes.
  • Pregnancy during treatment.
  • Locoregional relapse.
  • Certain EBV antibody titer patterns.

Small cancers of the nasopharynx are highly curable by radiation therapy, and patients with these small cancers have shown survival rates of 80% to 90%.[10]

Moderately advanced lesions without clinical evidence of spread to cervical lymph nodes are often curable, and patients with these lesions have shown survival rates of 50% to 70%.

Follow-up

Follow-up for patients includes the following:

  • Routine periodic examination of the original tumor site and neck.
  • CT or PET-CT scan.
  • MRI scan.
  • Blood work.
  • EBV titers.

Monitoring of patients should include the following:

  • Surveillance of thyroid and pituitary function.
  • Dental and oral hygiene.
  • Jaw exercises to avoid trismus.
  • Evaluation of cranial nerve function, especially as it relates to vision and hearing.
  • Evaluation of systemic complaints to identify distant metastasis.

Although most recurrences occur within 5 years of diagnosis, relapse can be seen at longer intervals. The incidence of second primary malignancies is less than after treatment of tumors at other head and neck sites.[11]

Poorly differentiated squamous cell cancer has been associated with EBV antibodies.[4][12] High-titer antibodies to virus capsid antigen and early antigen, especially of high IgA class, or high titers that persist after therapy, have been associated with a poorer prognosis.[13] This finding remains under evaluation.

Tumors of many histologies can occur in the nasopharynx, but this discussion, like the American Joint Committee on Cancer nasopharynx staging, refers exclusively to WHO grade I-, II-, and III-type nasopharyngeal carcinoma.

References:

  1. Chien YC, Chen JY, Liu MY, et al.: Serologic markers of Epstein-Barr virus infection and nasopharyngeal carcinoma in Taiwanese men. N Engl J Med 345 (26): 1877-82, 2001.

  2. Decker J, Goldstein JC: Risk factors in head and neck cancer. N Engl J Med 306 (19): 1151-5, 1982.

  3. Chen L, Gallicchio L, Boyd-Lindsley K, et al.: Alcohol consumption and the risk of nasopharyngeal carcinoma: a systematic review. Nutr Cancer 61 (1): 1-15, 2009.

  4. Feinmesser R, Miyazaki I, Cheung R, et al.: Diagnosis of nasopharyngeal carcinoma by DNA amplification of tissue obtained by fine-needle aspiration. N Engl J Med 326 (1): 17-21, 1992.

  5. Cummings CW, Fredrickson JM, Harker LA, et al.: Otolaryngology - Head and Neck Surgery. Saint Louis, Mo: Mosby-Year Book, Inc., 1998.

  6. Mendenhall WM, Werning JW, Pfister DG: Treatment of head and neck cancer. In: DeVita VT Jr, Lawrence TS, Rosenberg SA: Cancer: Principles and Practice of Oncology. 9th ed. Philadelphia, Pa: Lippincott Williams & Wilkins, 2011, pp 729-80.

  7. Laramore GE, ed.: Radiation Therapy of Head and Neck Cancer. Berlin: Springer-Verlag, 1989.

  8. Sanguineti G, Geara FB, Garden AS, et al.: Carcinoma of the nasopharynx treated by radiotherapy alone: determinants of local and regional control. Int J Radiat Oncol Biol Phys 37 (5): 985-96, 1997.

  9. Lee CC, Huang TT, Lee MS, et al.: Clinical application of tumor volume in advanced nasopharyngeal carcinoma to predict outcome. Radiat Oncol 5: 20, 2010.

  10. Bailet JW, Mark RJ, Abemayor E, et al.: Nasopharyngeal carcinoma: treatment results with primary radiation therapy. Laryngoscope 102 (9): 965-72, 1992.

  11. Cooper JS, Scott C, Marcial V, et al.: The relationship of nasopharyngeal carcinomas and second independent malignancies based on the Radiation Therapy Oncology Group experience. Cancer 67 (6): 1673-7, 1991.

  12. Neel HB 3rd, Pearson GR, Taylor WF: Antibodies to Epstein-Barr virus in patients with nasopharyngeal carcinoma and in comparison groups. Ann Otol Rhinol Laryngol 93 (5 Pt 1): 477-82, 1984 Sep-Oct.

  13. Lin JC, Chen KY, Wang WY, et al.: Detection of Epstein-Barr virus DNA the peripheral-blood cells of patients with nasopharyngeal carcinoma: relationship to distant metastasis and survival. J Clin Oncol 19 (10): 2607-15, 2001.

Cellular Classification of Nasopharyngeal Cancer

Although a wide variety of malignant tumors may arise in the nasopharynx, only squamous cell carcinoma is considered in this discussion because management of the other types varies substantially with histology. Subdivisions of squamous cell carcinoma in this site include the following:

World Health Organization (WHO) histopathological grading system describes three types of nasopharyngeal cancer:

  1. Keratinizing squamous cell carcinoma.
  2. Nonkeratinizing squamous cell carcinoma.
  3. Undifferentiated carcinoma (most common subtype).

Previous subdivisions of nasopharyngeal carcinoma included lymphoepithelioma, which is now classified as WHO grade III characterized by lymphoid infiltrate.[1]

WHO grade I-type cancer accounts for 20% of cases in United States and is associated with alcohol and tobacco use; WHO grade II and III represent the endemic form seen in Southern China.

The presence of keratin has been associated with reduced local control and survival.

References:

  1. Shanmugaratnam K, Sobin L: Histological Typing of Upper Respiratory Tract Tumours. Geneva: World Health Organization, 1978. International Histologic Classification of Tumours: No. 19.

Stage Information for Nasopharyngeal Cancer

Staging systems are all clinical staging and are based on the best possible estimate of the extent of disease before treatment.[1][2] Assessment of the primary tumor is based on inspection and palpation, and fiberoptic endoscopic evaluation. The tumor must be confirmed histologically, and any other pathologic data obtained on biopsy may be included. Evaluation of the function of the cranial nerves is especially appropriate for tumors of the nasopharynx. The appropriate nodal drainage areas are examined by careful palpation and radiologic evaluation. The retropharyngeal lymph nodes are the first echelon of drainage.[3][4] Information from diagnostic imaging studies may be used in staging. Magnetic resonance imaging provides additional information to computed tomographic scanning in the evaluation of skull base invasion and intracranial spread.[5] Positron emission tomography scans combined with CT are helpful in radiation treatment planning for target delineation of the primary tumor, aids in detection of metastatic nodal involvement and metastatic spread such as lung or skeletal metastases in patients with advanced nasopharyngeal cancer.[6]

If a patient has a relapse, a complete reassessment must be done to select the appropriate additional therapy.

Definitions of TNM

The American Joint Committee on Cancer (AJCC) has designated staging by TNM classification to define nasopharyngeal cancer.[7]

Table 1. Primary Tumor (T)a

TX

Primary tumor cannot be assessed.

T0

No evidence of primary tumor.

Tis

Carcinoma in situ.

T1

Tumor confined to the nasopharynx, or tumor extends to oropharynx and/or nasal cavity without parapharyngeal extension.b

T2

Tumor with parapharyngeal extension.b

T3

Tumor involves bony structures of skull base and/or paranasal sinuses.

T4

Tumor with intracranial extension and/or involvement of cranial nerves, hypopharynx, orbit, or with extension to the infratemporal fossa/masticator space.

aReprinted with permission from AJCC: Pharynx. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 41-56.

bParapharyngeal extension denotes posterolateral infiltration of tumor.

Table 2. Regional Lymph Nodes (N)a, b

NX

Regional lymph nodes cannot be assessed.

N0

No regional lymph node metastasis.

N1

Unilateral metastasis in cervical lymph node(s), ≤6 cm in greatest dimension, above the supraclavicular fossa, and/or unilateral or bilateral, retropharyngeal lymph nodes, ≤6 cm in greatest dimension.c

N2

Bilateral metastasis in cervical lymph node(s), ≤6 cm in greatest dimension, above the supraclavicular fossa.d

N3

Metastasis in a lymph node(s)c >6 cm and/or to supraclavicular fossa.d

N3a

>6 cm in dimension.

N3b

Extension to the supraclavicular fossa.d

aReprinted with permission from AJCC: Pharynx. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 44-56.

bThe distribution and the prognostic impact of regional lymph node spread from nasopharyngeal cancer, particularly of the undifferentiated type, are different from those of other head and neck mucosal cancers and justify the use of a different N classification scheme.

cMidline nodes are considered ipsilateral nodes.

dSupraclavicular zone or fossa is relevant to the staging of nasopharyngeal carcinoma and is the triangular region originally described by Ho. It is defined by three points: (1) the superior margin of the sternal end of the clavicle, (2) the superior margin of the lateral end of the clavicle, (3) the point where the neck meets the shoulder. Note that this would include caudal portions of levels IV and VB. All cases with lymph nodes (whole or part) in the fossa are considered N3b.

Table 3. Distant Metastasis (M)a

M0

No distant metastasis.

M1

Distant metastasis.

aReprinted with permission from AJCC: Pharynx. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 41-56.

Table 4. Anatomic Stage/Prognostic Groupsa

Stage

T

N

M

0

Tis

N0

M0

I

T1

N0

M0

II

T1

N1

M0

T2

N0

M0

T2

N1

M0

III

T1

N2

M0

T2

N2

M0

T3

N0

M0

T3

N1

M0

T3

N2

M0

IVA

T4

N0

M0

T4

N1

M0

T4

N2

M0

IVB

Any T

N3

M0

IVC

Any T

Any N

M1

aReprinted with permission from AJCC: Pharynx. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 41-56.

References:

  1. Teo PM, Leung SF, Yu P, et al.: A comparison of Ho's, International Union Against Cancer, and American Joint Committee stage classifications for nasopharyngeal carcinoma. Cancer 67 (2): 434-9, 1991.

  2. Lee AW, Foo W, Law SC, et al.: Staging of nasopharyngeal carcinoma: from Ho's to the new UICC system. Int J Cancer 84 (2): 179-87, 1999.

  3. Mendenhall WM, Werning JW, Pfister DG: Treatment of head and neck cancer. In: DeVita VT Jr, Lawrence TS, Rosenberg SA: Cancer: Principles and Practice of Oncology. 9th ed. Philadelphia, Pa: Lippincott Williams & Wilkins, 2011, pp 729-80.

  4. Laramore GE, ed.: Radiation Therapy of Head and Neck Cancer. Berlin: Springer-Verlag, 1989.

  5. Consensus conference. Magnetic resonance imaging. JAMA 259 (14): 2132-8, 1988.

  6. Liu FY, Chang JT, Wang HM, et al.: [18F]fluorodeoxyglucose positron emission tomography is more sensitive than skeletal scintigraphy for detecting bone metastasis in endemic nasopharyngeal carcinoma at initial staging. J Clin Oncol 24 (4): 599-604, 2006.

  7. Pharynx. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 41-56.

Treatment Option Overview

Standard treatments for patients with nasopharyngeal cancer include the following:

  • Radiation therapy alone.
  • Concurrent chemoradiation followed by adjuvant chemotherapy.
  • Surgery for residual nodal disease.
  • Chemotherapy alone for metastatic disease.

High-dose radiation therapy with chemotherapy is the primary treatment of nasopharyngeal cancer, both for the primary tumor site and the neck.[1] When feasible, surgery is usually reserved for nodes that fail to regress after radiation therapy or for nodal recurrence following clinical complete response. Radiation therapy dose and field margins are individually tailored to the location and size of the primary tumor and lymph nodes.[2][3][4][5] Although most tumors are treated with external-beam radiation therapy (EBRT) exclusively, in some tumors radiation therapy may be boosted with intracavitary or interstitial implants or by the use of stereotactic radiosurgery when clinical expertise is available, and the anatomy is suitable.[6][7][8][9][10] Intensity-modulated radiation therapy (IMRT) results in a lower incidence of xerostomia and may provide a better quality of life than conventional three-dimensional or two-dimensional radiation therapy.[11][12][Level of evidence: 1iiC] Results of a phase II RTOG study (RTOG-0225) showed the feasibility of IMRT in a multi-institutional setting and minimal grade III and IV xerostomia rates.[13] The rate of grade 2 xerostomia at 1 year from start of IMRT was 13.5%. Only 2 of 68 patients were reported with grade 3 xerostomia, and none had grade 4 xerostomia.[13][Level of evidence: 2C]

Accumulating evidence has demonstrated a high incidence (>30%–40%) of hypothyroidism in patients who have received radiation therapy that delivered EBRT to the entire thyroid gland or to the pituitary gland. Thyroid-function testing of patients should be considered prior to therapy and as part of posttreatment follow-up.[14][15]

Treatments under clinical evaluation for patients with nasopharyngeal cancer include the following:

  • Dose escalation with new radiation therapy techniques such as stereotactic radiation therapy boost.[16][Level of evidence: 3iiiDiv]
  • Brachytherapy.[17][Level of evidence: 3iiiDii]

Information about ongoing clinical trials is available from the NCI Web site.

References:

  1. Baujat B, Audry H, Bourhis J, et al.: Chemotherapy in locally advanced nasopharyngeal carcinoma: an individual patient data meta-analysis of eight randomized trials and 1753 patients. Int J Radiat Oncol Biol Phys 64 (1): 47-56, 2006.

  2. Perez CA, Devineni VR, Marcial-Vega V, et al.: Carcinoma of the nasopharynx: factors affecting prognosis. Int J Radiat Oncol Biol Phys 23 (2): 271-80, 1992.

  3. Lee AW, Law SC, Foo W, et al.: Nasopharyngeal carcinoma: local control by megavoltage irradiation. Br J Radiol 66 (786): 528-36, 1993.

  4. Geara FB, Sanguineti G, Tucker SL, et al.: Carcinoma of the nasopharynx treated by radiotherapy alone: determinants of distant metastasis and survival. Radiother Oncol 43 (1): 53-61, 1997.

  5. Sanguineti G, Geara FB, Garden AS, et al.: Carcinoma of the nasopharynx treated by radiotherapy alone: determinants of local and regional control. Int J Radiat Oncol Biol Phys 37 (5): 985-96, 1997.

  6. Mendenhall WM, Werning JW, Pfister DG: Treatment of head and neck cancer. In: DeVita VT Jr, Lawrence TS, Rosenberg SA: Cancer: Principles and Practice of Oncology. 9th ed. Philadelphia, Pa: Lippincott Williams & Wilkins, 2011, pp 729-80.

  7. Itami J, Anzai Y, Nemoto K, et al.: Prognostic factors for local control in nasopharyngeal cancer (NPC): analysis by multivariate proportional hazard models. Radiother Oncol 21 (4): 233-9, 1991.

  8. Levendag PC, Schmitz PI, Jansen PP, et al.: Fractionated high-dose-rate brachytherapy in primary carcinoma of the nasopharynx. J Clin Oncol 16 (6): 2213-20, 1998.

  9. Teo PM, Leung SF, Lee WY, et al.: Intracavitary brachytherapy significantly enhances local control of early T-stage nasopharyngeal carcinoma: the existence of a dose-tumor-control relationship above conventional tumoricidal dose. Int J Radiat Oncol Biol Phys 46 (2): 445-58, 2000.

  10. Le QT, Tate D, Koong A, et al.: Improved local control with stereotactic radiosurgical boost in patients with nasopharyngeal carcinoma. Int J Radiat Oncol Biol Phys 56 (4): 1046-54, 2003.

  11. Pow EH, Kwong DL, McMillan AS, et al.: Xerostomia and quality of life after intensity-modulated radiotherapy vs. conventional radiotherapy for early-stage nasopharyngeal carcinoma: initial report on a randomized controlled clinical trial. Int J Radiat Oncol Biol Phys 66 (4): 981-91, 2006.

  12. Kam MK, Leung SF, Zee B, et al.: Prospective randomized study of intensity-modulated radiotherapy on salivary gland function in early-stage nasopharyngeal carcinoma patients. J Clin Oncol 25 (31): 4873-9, 2007.

  13. Lee N, Harris J, Garden AS, et al.: Intensity-modulated radiation therapy with or without chemotherapy for nasopharyngeal carcinoma: radiation therapy oncology group phase II trial 0225. J Clin Oncol 27 (22): 3684-90, 2009.

  14. Turner SL, Tiver KW, Boyages SC: Thyroid dysfunction following radiotherapy for head and neck cancer. Int J Radiat Oncol Biol Phys 31 (2): 279-83, 1995.

  15. Constine LS: What else don't we know about the late effects of radiation in patients treated for head and neck cancer? Int J Radiat Oncol Biol Phys 31 (2): 427-9, 1995.

  16. Tate DJ, Adler JR Jr, Chang SD, et al.: Stereotactic radiosurgical boost following radiotherapy in primary nasopharyngeal carcinoma: impact on local control. Int J Radiat Oncol Biol Phys 45 (4): 915-21, 1999.

  17. Lu JJ, Shakespeare TP, Tan LK, et al.: Adjuvant fractionated high-dose-rate intracavitary brachytherapy after external beam radiotherapy in Tl and T2 nasopharyngeal carcinoma. Head Neck 26 (5): 389-95, 2004.

Stage I Nasopharyngeal Cancer

Standard treatment options:

  • High-dose radiation therapy to the primary tumor site and prophylactic radiation therapy to the nodal drainage.[1]

Current Clinical Trials

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage I nasopharyngeal cancer. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

References:

  1. Xiao WW, Han F, Lu TX, et al.: Treatment outcomes after radiotherapy alone for patients with early-stage nasopharyngeal carcinoma. Int J Radiat Oncol Biol Phys 74 (4): 1070-6, 2009.

Stage II Nasopharyngeal Cancer

Standard treatment options:

  1. Chemoradiation therapy followed by adjuvant chemotherapy, as was used in the INT-0099 trial, for example.[1][Level of evidence: 3iiiA] (Patients with parapharyngeal extension were originally staged as T3 in the INT-0099 study and are now considered T2 in the current staging.)
  2. High-dose radiation therapy to the primary tumor site and prophylactic radiation therapy to the nodal drainage.[2]

Current Clinical Trials

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage II nasopharyngeal cancer. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

References:

  1. Cheng SH, Tsai SY, Yen KL, et al.: Concomitant radiotherapy and chemotherapy for early-stage nasopharyngeal carcinoma. J Clin Oncol 18 (10): 2040-5, 2000.

  2. Xiao WW, Han F, Lu TX, et al.: Treatment outcomes after radiotherapy alone for patients with early-stage nasopharyngeal carcinoma. Int J Radiat Oncol Biol Phys 74 (4): 1070-6, 2009.

Stage III Nasopharyngeal Cancer

Standard treatment options:

  1. Combined chemoradiation therapy.[1][2]
  2. Combined chemoradiation therapy followed by adjuvant chemotherapy, as evidenced in INT-0099, for example.[3][4][2][5][6][7][8][9][10][11][12][13][14]
  3. Altered fractionation radiation therapy.[15][16]
  4. Neck dissection may be indicated for persistent or recurrent nodes if the primary tumor site is controlled.[17]

Treatment options under clinical evaluation:

  • Neoadjuvant chemotherapy. Neoadjuvant chemotherapy as given in clinical trials has been used to shrink tumors, which renders them more definitively treatable with radiation therapy. Chemotherapy is given prior to the other modalities, hence the designation neoadjuvant to distinguish it from standard adjuvant therapy, which is given after or during definitive therapy with radiation or after surgery. Many drug combinations have been used in neoadjuvant chemotherapy.

    Two randomized, prospective trials compared combination chemotherapy (i.e., cisplatin, epirubicin, and bleomycin or cisplatin plus fluorouracil [5-FU] infusion) plus radiation therapy to radiation therapy alone.[3][Level of evidence: 1iiA];[18][Level of evidence: 1iiDii] Although disease-free survival was improved in the chemotherapy group for both groups, improvement in overall survival was reported only from the Intergroup trial in which chemotherapy with cisplatin was ever concurrently given.[3]

Clinical trials for advanced tumors evaluating the use of chemotherapy before radiation therapy, concomitant with radiation therapy, or as adjuvant therapy after radiation therapy should be considered.[19][20][21][22]

Current Clinical Trials

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage III nasopharyngeal cancer. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

References:

  1. Langendijk JA, Leemans ChR, Buter J, et al.: The additional value of chemotherapy to radiotherapy in locally advanced nasopharyngeal carcinoma: a meta-analysis of the published literature. J Clin Oncol 22 (22): 4604-12, 2004.

  2. Huncharek M, Kupelnick B: Combined chemoradiation versus radiation therapy alone in locally advanced nasopharyngeal carcinoma: results of a meta-analysis of 1,528 patients from six randomized trials. Am J Clin Oncol 25 (3): 219-23, 2002.

  3. Al-Sarraf M, LeBlanc M, Giri PG, et al.: Chemoradiotherapy versus radiotherapy in patients with advanced nasopharyngeal cancer: phase III randomized Intergroup study 0099. J Clin Oncol 16 (4): 1310-7, 1998.

  4. Teo PM, Chan AT, Lee WY, et al.: Enhancement of local control in locally advanced node-positive nasopharyngeal carcinoma by adjunctive chemotherapy. Int J Radiat Oncol Biol Phys 43 (2): 261-71, 1999.

  5. Chan AT, Teo PM, Ngan RK, et al.: Concurrent chemotherapy-radiotherapy compared with radiotherapy alone in locoregionally advanced nasopharyngeal carcinoma: progression-free survival analysis of a phase III randomized trial. J Clin Oncol 20 (8): 2038-44, 2002.

  6. Chua DT, Ma J, Sham JS, et al.: Long-term survival after cisplatin-based induction chemotherapy and radiotherapy for nasopharyngeal carcinoma: a pooled data analysis of two phase III trials. J Clin Oncol 23 (6): 1118-24, 2005.

  7. Wee J, Tan EH, Tai BC, et al.: Randomized trial of radiotherapy versus concurrent chemoradiotherapy followed by adjuvant chemotherapy in patients with American Joint Committee on Cancer/International Union against cancer stage III and IV nasopharyngeal cancer of the endemic variety. J Clin Oncol 23 (27): 6730-8, 2005.

  8. Zhang L, Zhao C, Peng PJ, et al.: Phase III study comparing standard radiotherapy with or without weekly oxaliplatin in treatment of locoregionally advanced nasopharyngeal carcinoma: preliminary results. J Clin Oncol 23 (33): 8461-8, 2005.

  9. Baujat B, Audry H, Bourhis J, et al.: Chemotherapy in locally advanced nasopharyngeal carcinoma: an individual patient data meta-analysis of eight randomized trials and 1753 patients. Int J Radiat Oncol Biol Phys 64 (1): 47-56, 2006.

  10. Baujat B, Audry H, Bourhis J, et al.: Chemotherapy as an adjunct to radiotherapy in locally advanced nasopharyngeal carcinoma. Cochrane Database Syst Rev (4): CD004329, 2006.

  11. Chen Y, Liu MZ, Liang SB, et al.: Preliminary results of a prospective randomized trial comparing concurrent chemoradiotherapy plus adjuvant chemotherapy with radiotherapy alone in patients with locoregionally advanced nasopharyngeal carcinoma in endemic regions of china. Int J Radiat Oncol Biol Phys 71 (5): 1356-64, 2008.

  12. Lee AW, Tung SY, Chua DT, et al.: Randomized trial of radiotherapy plus concurrent-adjuvant chemotherapy vs radiotherapy alone for regionally advanced nasopharyngeal carcinoma. J Natl Cancer Inst 102 (15): 1188-98, 2010.

  13. Lee AW, Tung SY, Chan AT, et al.: A randomized trial on addition of concurrent-adjuvant chemotherapy and/or accelerated fractionation for locally-advanced nasopharyngeal carcinoma. Radiother Oncol 98 (1): 15-22, 2011.

  14. Lee AW, Tung SY, Ngan RK, et al.: Factors contributing to the efficacy of concurrent-adjuvant chemotherapy for locoregionally advanced nasopharyngeal carcinoma: combined analyses of NPC-9901 and NPC-9902 Trials. Eur J Cancer 47 (5): 656-66, 2011.

  15. Johnson CR, Schmidt-Ullrich RK, Wazer DE: Concomitant boost technique using accelerated superfractionated radiation therapy for advanced squamous cell carcinoma of the head and neck. Cancer 69 (11): 2749-54, 1992.

  16. Chen CY, Han F, Zhao C, et al.: Treatment results and late complications of 556 patients with locally advanced nasopharyngeal carcinoma treated with radiotherapy alone. Br J Radiol 82 (978): 452-8, 2009.

  17. Mendenhall WM, Werning JW, Pfister DG: Treatment of head and neck cancer. In: DeVita VT Jr, Lawrence TS, Rosenberg SA: Cancer: Principles and Practice of Oncology. 9th ed. Philadelphia, Pa: Lippincott Williams & Wilkins, 2011, pp 729-80.

  18. Preliminary results of a randomized trial comparing neoadjuvant chemotherapy (cisplatin, epirubicin, bleomycin) plus radiotherapy vs. radiotherapy alone in stage IV(> or = N2, M0) undifferentiated nasopharyngeal carcinoma: a positive effect on progression-free survival. International Nasopharynx Cancer Study Group. VUMCA I trial. Int J Radiat Oncol Biol Phys 35 (3): 463-9, 1996.

  19. Azli N, Armand JP, Rahal M, et al.: Alternating chemo-radiotherapy with cisplatin and 5-fluorouracil plus bleomycin by continuous infusion for locally advanced undifferentiated carcinoma nasopharyngeal type. Eur J Cancer 28A (11): 1792-7, 1992.

  20. Chan AT, Teo PM, Leung TW, et al.: A prospective randomized study of chemotherapy adjunctive to definitive radiotherapy in advanced nasopharyngeal carcinoma. Int J Radiat Oncol Biol Phys 33 (3): 569-77, 1995.

  21. Merlano M, Benasso M, Corvò R, et al.: Five-year update of a randomized trial of alternating radiotherapy and chemotherapy compared with radiotherapy alone in treatment of unresectable squamous cell carcinoma of the head and neck. J Natl Cancer Inst 88 (9): 583-9, 1996.

  22. Jeremic B, Shibamoto Y, Milicic B, et al.: Hyperfractionated radiation therapy with or without concurrent low-dose daily cisplatin in locally advanced squamous cell carcinoma of the head and neck: a prospective randomized trial. J Clin Oncol 18 (7): 1458-64, 2000.

Stage IV Nasopharyngeal Cancer

Standard treatment options:

  1. Chemoradiation therapy followed by adjuvant chemotherapy, as evidenced in INT-0099, for example.[1][2][3][4][5][6][7][8][9][10][11][12][13][14]
  2. Altered fractionation including hyperfractionated radiation therapy.[15][16]
  3. Neck dissection should be reserved for persistent or recurrent nodes.[17]
  4. Chemotherapy for patients with stage IVC disease.[18]

Treatment options under clinical evaluation:

  1. Neoadjuvant chemotherapy. Neoadjuvant chemotherapy has been used to shrink tumors, which renders them more definitively treatable with radiation therapy. Chemotherapy is given prior to the other modalities, hence the designation neoadjuvant to distinguish it from standard adjuvant therapy, which is given after or during definitive therapy with radiation or after surgery. Many drug combinations have been used in neoadjuvant chemotherapy.

    Clinical trials for advanced tumors to evaluate the use of chemotherapy before radiation therapy, concomitant with radiation therapy, or as adjuvant therapy after radiation therapy should be considered.[19][20][21][22]

    A phase II, randomized study of 65 patients with stage III and IV nasopharyngeal carcinoma were randomly assigned to neoadjuvant docetaxel (75 mg/m2) and cisplatin (75 mg/m2) every 3 weeks for two cycles followed by cisplatin (40 mg/m2) every week versus chemoradiation alone. Rates of grade 3 or 4 neutropenia were 97% during the neoadjuvant arm with no difference in toxicities between the two groups during the chemoradiation portion of treatment. The 3-year progression-free survival for neoadjuvant docetaxel versus the control arm was 88.2% and 59.5% (hazard ratio [HR], 0.49; 95% confidence interval [CI], 0.20–1.19; P = .12). The 3-year overall survival for neoadjuvant docetaxel versus the control arm was 94.1% and 67.7% (HR, 0.24; 95% CI, 0.078–0.73; P = .012).[23][Level of evidence: 1iiDiii] These data have to be confirmed in a definitive phase III trial.

    Three randomized, prospective trials compared combination chemotherapy (i.e., cisplatin, epirubicin, and bleomycin or cisplatin plus fluorouracil [5-FU] infusion) plus radiation therapy to radiation therapy alone.[1][Level of evidence: 1iiA]; [24][25][Level of evidence: 1iiDii] Although disease-free survival (DFS) was improved in the chemotherapy group for both groups, improvement in overall survival (OS) was reported only from the Intergroup trial in which chemotherapy with cisplatin was ever concurrently given.[1]

  2. Concurrent radiation therapy with chemotherapy. A study of 1,355 patients compared concurrent radiation therapy with carboplatin or cisplatin administered with 96-hour infusion of 5-FU monthly for three cycles.[26] The 3-year DFS rate was 63.4% for patients in the cisplatin arm and 60.9% for patients in the carboplatin arm (P = .961; HR, 0.70; 95% CI, 0.50–0.98). OS rates were 77% for patients in the cisplatin arm and 79% for patients in the carboplatin arm (P = .988; HR, 0.83; 95% CI, 0.63–1.010).[26][Level of evidence: 1iiA] Toxicity to kidneys and red blood cell count was greater in patients in the cisplatin group.

Current Clinical Trials

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage IV nasopharyngeal cancer. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

References:

  1. Al-Sarraf M, LeBlanc M, Giri PG, et al.: Chemoradiotherapy versus radiotherapy in patients with advanced nasopharyngeal cancer: phase III randomized Intergroup study 0099. J Clin Oncol 16 (4): 1310-7, 1998.

  2. Teo PM, Chan AT, Lee WY, et al.: Enhancement of local control in locally advanced node-positive nasopharyngeal carcinoma by adjunctive chemotherapy. Int J Radiat Oncol Biol Phys 43 (2): 261-71, 1999.

  3. Chan AT, Teo PM, Ngan RK, et al.: Concurrent chemotherapy-radiotherapy compared with radiotherapy alone in locoregionally advanced nasopharyngeal carcinoma: progression-free survival analysis of a phase III randomized trial. J Clin Oncol 20 (8): 2038-44, 2002.

  4. Huncharek M, Kupelnick B: Combined chemoradiation versus radiation therapy alone in locally advanced nasopharyngeal carcinoma: results of a meta-analysis of 1,528 patients from six randomized trials. Am J Clin Oncol 25 (3): 219-23, 2002.

  5. Lin JC, Jan JS, Hsu CY, et al.: Phase III study of concurrent chemoradiotherapy versus radiotherapy alone for advanced nasopharyngeal carcinoma: positive effect on overall and progression-free survival. J Clin Oncol 21 (4): 631-7, 2003.

  6. Chua DT, Ma J, Sham JS, et al.: Long-term survival after cisplatin-based induction chemotherapy and radiotherapy for nasopharyngeal carcinoma: a pooled data analysis of two phase III trials. J Clin Oncol 23 (6): 1118-24, 2005.

  7. Wee J, Tan EH, Tai BC, et al.: Randomized trial of radiotherapy versus concurrent chemoradiotherapy followed by adjuvant chemotherapy in patients with American Joint Committee on Cancer/International Union against cancer stage III and IV nasopharyngeal cancer of the endemic variety. J Clin Oncol 23 (27): 6730-8, 2005.

  8. Zhang L, Zhao C, Peng PJ, et al.: Phase III study comparing standard radiotherapy with or without weekly oxaliplatin in treatment of locoregionally advanced nasopharyngeal carcinoma: preliminary results. J Clin Oncol 23 (33): 8461-8, 2005.

  9. Baujat B, Audry H, Bourhis J, et al.: Chemotherapy in locally advanced nasopharyngeal carcinoma: an individual patient data meta-analysis of eight randomized trials and 1753 patients. Int J Radiat Oncol Biol Phys 64 (1): 47-56, 2006.

  10. Baujat B, Audry H, Bourhis J, et al.: Chemotherapy as an adjunct to radiotherapy in locally advanced nasopharyngeal carcinoma. Cochrane Database Syst Rev (4): CD004329, 2006.

  11. Chen Y, Liu MZ, Liang SB, et al.: Preliminary results of a prospective randomized trial comparing concurrent chemoradiotherapy plus adjuvant chemotherapy with radiotherapy alone in patients with locoregionally advanced nasopharyngeal carcinoma in endemic regions of china. Int J Radiat Oncol Biol Phys 71 (5): 1356-64, 2008.

  12. Lee AW, Tung SY, Chua DT, et al.: Randomized trial of radiotherapy plus concurrent-adjuvant chemotherapy vs radiotherapy alone for regionally advanced nasopharyngeal carcinoma. J Natl Cancer Inst 102 (15): 1188-98, 2010.

  13. Lee AW, Tung SY, Chan AT, et al.: A randomized trial on addition of concurrent-adjuvant chemotherapy and/or accelerated fractionation for locally-advanced nasopharyngeal carcinoma. Radiother Oncol 98 (1): 15-22, 2011.

  14. Lee AW, Tung SY, Ngan RK, et al.: Factors contributing to the efficacy of concurrent-adjuvant chemotherapy for locoregionally advanced nasopharyngeal carcinoma: combined analyses of NPC-9901 and NPC-9902 Trials. Eur J Cancer 47 (5): 656-66, 2011.

  15. Johnson CR, Schmidt-Ullrich RK, Wazer DE: Concomitant boost technique using accelerated superfractionated radiation therapy for advanced squamous cell carcinoma of the head and neck. Cancer 69 (11): 2749-54, 1992.

  16. Chen CY, Han F, Zhao C, et al.: Treatment results and late complications of 556 patients with locally advanced nasopharyngeal carcinoma treated with radiotherapy alone. Br J Radiol 82 (978): 452-8, 2009.

  17. Mendenhall WM, Werning JW, Pfister DG: Treatment of head and neck cancer. In: DeVita VT Jr, Lawrence TS, Rosenberg SA: Cancer: Principles and Practice of Oncology. 9th ed. Philadelphia, Pa: Lippincott Williams & Wilkins, 2011, pp 729-80.

  18. Ma BB, Tannock IF, Pond GR, et al.: Chemotherapy with gemcitabine-containing regimens for locally recurrent or metastatic nasopharyngeal carcinoma. Cancer 95 (12): 2516-23, 2002.

  19. Dimery IW, Peters LJ, Goepfert H, et al.: Effectiveness of combined induction chemotherapy and radiotherapy in advanced nasopharyngeal carcinoma. J Clin Oncol 11 (10): 1919-28, 1993.

  20. Chan AT, Teo PM, Leung TW, et al.: A prospective randomized study of chemotherapy adjunctive to definitive radiotherapy in advanced nasopharyngeal carcinoma. Int J Radiat Oncol Biol Phys 33 (3): 569-77, 1995.

  21. Merlano M, Benasso M, Corvò R, et al.: Five-year update of a randomized trial of alternating radiotherapy and chemotherapy compared with radiotherapy alone in treatment of unresectable squamous cell carcinoma of the head and neck. J Natl Cancer Inst 88 (9): 583-9, 1996.

  22. Jeremic B, Shibamoto Y, Milicic B, et al.: Hyperfractionated radiation therapy with or without concurrent low-dose daily cisplatin in locally advanced squamous cell carcinoma of the head and neck: a prospective randomized trial. J Clin Oncol 18 (7): 1458-64, 2000.

  23. Hui EP, Ma BB, Leung SF, et al.: Randomized phase II trial of concurrent cisplatin-radiotherapy with or without neoadjuvant docetaxel and cisplatin in advanced nasopharyngeal carcinoma. J Clin Oncol 27 (2): 242-9, 2009.

  24. Preliminary results of a randomized trial comparing neoadjuvant chemotherapy (cisplatin, epirubicin, bleomycin) plus radiotherapy vs. radiotherapy alone in stage IV(> or = N2, M0) undifferentiated nasopharyngeal carcinoma: a positive effect on progression-free survival. International Nasopharynx Cancer Study Group. VUMCA I trial. Int J Radiat Oncol Biol Phys 35 (3): 463-9, 1996.

  25. Lee AW, Lau WH, Tung SY, et al.: Preliminary results of a randomized study on therapeutic gain by concurrent chemotherapy for regionally-advanced nasopharyngeal carcinoma: NPC-9901 Trial by the Hong Kong Nasopharyngeal Cancer Study Group. J Clin Oncol 23 (28): 6966-75, 2005.

  26. Chitapanarux I, Lorvidhaya V, Kamnerdsupaphon P, et al.: Chemoradiation comparing cisplatin versus carboplatin in locally advanced nasopharyngeal cancer: randomised, non-inferiority, open trial. Eur J Cancer 43 (9): 1399-406, 2007.

Recurrent Nasopharyngeal Cancer

Standard treatment options:

  1. Selected patients with local recurrence may be retreated with moderate-dose external-beam radiation therapy using intensity-modulated radiation therapy, stereotactic radiation therapy, or intracavitary or interstitial radiation to the site of recurrence.[1][2][3]
  2. In highly selected patients, surgical resection of locally recurrent lesions may be considered.
  3. If a patient has metastatic disease or local recurrence that is no longer amenable to surgery or radiation therapy, chemotherapy should be considered.[4][5][6]

Treatment options under clinical evaluation:

  • Clinical trials evaluating chemotherapy should be considered.
  • Stereotactic radiation for locally recurrent disease or persistence.[7][8][9][Level of evidence: 3iiiDiv]

Current Clinical Trials

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent nasopharyngeal cancer. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

References:

  1. Mendenhall WM, Werning JW, Pfister DG: Treatment of head and neck cancer. In: DeVita VT Jr, Lawrence TS, Rosenberg SA: Cancer: Principles and Practice of Oncology. 9th ed. Philadelphia, Pa: Lippincott Williams & Wilkins, 2011, pp 729-80.

  2. Vikram B, Strong EW, Shah JP, et al.: Intraoperative radiotherapy in patients with recurrent head and neck cancer. Am J Surg 150 (4): 485-7, 1985.

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This information is provided by the National Cancer Institute.

This information was last updated on July 31, 2014.

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