Nasopharyngeal Cancer

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What is Nasopharyngeal Cancer? 

Cancer begins when cells in the body become abnormal and multiply without control or order. These cells form a growth of tissue, called a tumor. A tumor can be benign (noncancerous) or malignant (cancerous).

Nasopharyngeal cancer (also called nasopharyngeal carcinoma or NPC) is a disease of the nasopharynx, which is the air passageway at the upper part of the pharynx (throat) behind the nose. The pharynx is a hollow tube approximately five inches long that starts behind the nose and ends on top of the larynx (or voice box) and esophagus (the swallowing tube that goes from the throat to the stomach). The nostrils lead through the nasal cavity into the nasopharynx, and an opening on each side of the nasopharynx (called the Eustachian tube opening) leads into the middle ear on each side.

Types of nasopharyngeal tumors

There are several types of benign nasopharyngeal tumors, including angiofibromas and hemangiomas that involve the vascular (blood-carrying) system and tumors in the lining of the nasopharynx that include the minor salivary glands.

A malignant nasopharyngeal tumor is cancerous. This means it can invade and damage healthy tissues and organs in other parts of the body. The nasopharynx contains several types of tissue, and each contains several types of cells. Different cancers can develop in each kind of cell. The differences are important because they determine the seriousness of the cancer and the type of treatment needed. According to the World Health Organization (WHO), NPC is classified into three subtypes:

Keratinizing squamous cell carcinoma (WHO type 1)

Nonkeratinizing squamous cell carcinoma (WHO type 2)

Undifferentiated or poorly differentiated carcinoma, including lymphoepithelioma and anaplastic variants (WHO type 3). Many types of nasopharyngeal cancer contain white blood cells, and these lymphocytes give it the name of lymphoepithelioma. (See more details about differentiation in the Staging section.)

NPC is one of five main types of cancer in the head and neck region, a grouping called head and neck cancer.

Risk Factors and Prevention

A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors often influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do. However, knowing your risk factors and talking about them with your doctor may help you make more informed lifestyle and health care choices.

Two risk factors greatly increase the risk of NPC:

Tobacco use. Use of tobacco (including cigarettes, cigars, pipes, chewing tobacco, and snuff) is the single greatest risk factor for head and neck cancer. Smokers with NPC are most likely to have the squamous cell type.

Alcohol. Frequent and heavy consumption of alcohol is a risk factor for head and neck cancer.

Eighty-five percent (85%) of head and neck cancer is linked to tobacco use. Using alcohol and tobacco together increases this risk even more, although the influence of this in NPC is less than for other head and neck cancers. Recent research suggests that people who have used marijuana may be at higher risk for head and neck cancer. Secondhand smoke may also increase a person’s risk of head and neck cancer.

Other factors that can raise a person’s risk of NPC include the following:

Geography/ancestry. NPC is most common in people who live in Southeast China and Hong Kong. When people move away from high-risk areas of the world to countries where NPC is less common, subsequent generations of their family have a gradual reduction in their inherited risk of NPC.

Epstein-Barr virus (EBV). Exposure to EBV, which is more commonly known as the virus that causes mononucleosis (or “mono”), plays a role in causing nasopharyngeal cancer to develop.

Gender. Men are two times more likely than women to develop NPC.

Age. The risk of NPC increases as a person gets older. However, about half of the people with nasopharyngeal cancer in the United States are younger than 55. A person of any age can be diagnosed with NPC.

Diet. Eating large amounts of salt-cured fish and meats on a regular basis increase the risk of NPC.

Environmental exposure. Extensive exposure to dust and smoke may increase the risk of NPC.

Prevention

Although some of the risk factors of NPC cannot be controlled, such as age, several can be avoided by making lifestyle changes. Stopping the use of all tobacco products is the most important thing a person can do to reduce the risk of NPC, even for people who have been smoking for many years.

Symptoms and Signs :  People with NPC may experience the following symptoms or signs. Sometimes, people with NPC do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom or sign on this list, please talk with your doctor.         

A lump in the neck (the most common symptom)

Nasal obstruction or stuffiness

Trouble hearing or hearing loss and/or a sense of fullness or pain in the ear that is caused by a buildup of fluid in the middle ear (serous otitis media, caused by blockage of the Eustachian tube), especially if persistent and occurring in just one ear

Pain and ringing in the ear

A persistent sore throat

Trouble breathing or speaking

Frequent nose bleeds

Pain, numbness, or paralysis in the face

Frequent headaches

Difficulty opening the mouth

Blurred or double vision

Fatigue

Unexplained weight loss

People who notice any of these warning signs should talk with a doctor and/or dentist right away. When detected early, cancers of the head and neck have a much better chance of cure.

Your doctor will ask you questions about the symptoms you are experiencing to help find out the cause of the problem, called a diagnosis. This may include how long you’ve been experiencing the symptom(s) and how often.

Because many of these symptoms can be caused by other noncancerous conditions as well, it is important to receive regular health and dental screenings; this is particularly important for people who routinely drink alcohol or currently use tobacco products or have used them in the past.

In fact, people who use alcohol and tobacco should receive a general screening examination at least once a year. This is a simple, quick procedure in which the doctor looks in the nose, mouth, and throat for abnormalities and feels for lumps in the neck. If anything unusual is found, then the doctor will recommend a more extensive examination using one or more of the diagnostic procedures mentioned inDiagnosis.

If cancer is diagnosed, relieving symptoms and side effects remains an important part of cancer care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your health care team about symptoms you experience, including any new symptoms or a change in symptoms.

Diagnosis : Doctors use many tests to diagnose cancer and find out if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For most types of cancer, a biopsy is the only way to make a definitive diagnosis of cancer. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether the cancer has metastasized. Your doctor may consider these factors when choosing a diagnostic test:

Age and medical condition

Type of cancer suspected

Severity of symptoms

Previous test results

The following tests may be used to diagnose NPC:

Physical examination and blood test. The doctor feels for any lumps on the neck, lips, gums, and cheeks. Also, the doctor will inspect the nose, mouth, throat, and tongue for abnormalities, often using a light and/or mirror for a clearer view. A blood test to check for antibodies against the EBV virus may be done at the same time.

Endoscopy. This test allows the doctor to see inside the body with a thin, lighted, flexible tube called an endoscope. The person may be sedated as the tube is inserted through the mouth or nose to examine the head and neck areas. When an endoscopy is done to look into the nasopharynx, it is called a nasopharyngoscopy.

Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. The sample removed from the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease). The type of biopsy performed will depend on the location of the cancer. In a fine needle aspiration biopsy, cells are withdrawn using a thin needle inserted directly into the tumor. The cells are examined under a microscope for signs of cancer (called cytologic examination). The biopsy may be performed using local anesthesia (medication to block awareness of pain) or general anesthesia.

X-ray. An x-ray is a way to create a picture of the structures inside of the body, using a small amount of radiation. Sometimes, a barium swallow may be required before having an x-ray. The barium coats the mouth and throat to enhance the image on the x-ray. An x-ray of the skull and chest may be needed to learn more about the extent of NPC.

Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. Sometimes, a contrast medium (a special dye) is injected into a patient’s vein to provide better detail.

Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body, especially images of soft tissue, such as the tonsils and base of the tongue. An MRI is more sensitive than a CT scan in detecting a tumor of the nasopharynx and its possible spread to nearby tissues or lymph nodes. A contrast medium may be injected into a patient’s vein to create a clearer picture.

Ultrasound. An ultrasound uses sound waves to create a picture of the internal organs.

Bone scan. A bone scan uses a radioactive tracer to look at the inside of the bones. The tracer is injected into a patient’s vein. It collects in areas of the bone and is detected by a special camera. Healthy bone appears gray to the camera, and areas of injury, such as those caused by cancer, appear dark.

Neurologic tests. These examinations involve the doctor testing nerve function, especially tactile sensation of the face and motor function of certain nerves in the head and neck area.

Hearing test. The doctor may perform a hearing test if he or she suspects there is fluid in the middle ear.

Positron emission tomography (PET) scan. A PET scan is a way to create pictures of organs and tissues inside the body. A small amount of a radioactive substance is injected into a patient’s body. This substance is absorbed mainly by organs and tissues that use the most energy. Because cancer tends to use energy actively, it absorbs more of the radioactive substance. A scanner then detects this substance to produce images of the inside of the body.

Staging with Illustrations : Staging is a way of describing where the cancer is located, if or where it has spread, and whether it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the cancer’s stage, so staging may not be complete until all of the tests are finished. Knowing the stage and histologic grade of cancer helps the doctor to decide what kind of treatment is to be given and can help predict a patient’s prognosis (chance of recovery). There are different stage descriptions for different types of cancer.

One tool that doctors use to describe the stage is the TNM system. This system judges three factors: the tumor itself, the lymph nodes around the tumor, and if the tumor has spread to other parts of the body. The results are combined to determine the stage of cancer for each person. There are five stages: stage 0 (zero) and stages I through IV (one through four). The stage provides a common way of describing the cancer so doctors can work together to plan the best treatments.

TNM is an abbreviation for tumor (T), node (N), and metastasis (M). Doctors look at these three factors to determine the stage of cancer: 

How large is the primary tumor, and where is it located? (Tumor, T)

Has the tumor spread to the lymph nodes? (Node, N)

Has the cancer metastasized (spread) to other parts of the body? (Metastasis, M)

Tumor. Using the TNM system, the "T" plus a letter or number (0 to 4) is used to describe the size and location of the tumor in NPC. Some stages are also divided into smaller groups that help describe the tumor in even more detail. Specific tumor stage information is listed below.

TX: The primary tumor cannot be evaluated.

T0: No evidence of a tumor is found.

Tis: This describes a stage called carcinoma (cancer) in situ. This is a very early cancer where cancer cells are found only in one layer of tissue.

T1: The tumor has not spread beyond the nasopharynx.

T2: The tumor extends into the soft tissue of the middle throat.

T3: The tumor extends into bony structure or into the area behind the nose.

T4: This describes a tumor that extends inside the head to the area of the brain or into the lower part of the throat.

Node. The “N” in the TNM staging system is for lymph nodes, the tiny, bean-shaped organs that help fight infection. Lymph nodes near where the cancer started are called regional lymph nodes. Lymph nodes in other parts of the body are called distant lymph nodes.

In people with NPC, doctors examine lymph nodes in a triangle-shaped area formed by three points: where the neck meets the shoulder, where the collarbone joins the tip of the shoulder, and where the front half of the collarbone meets the base of the neck.

NX: The regional lymph nodes cannot be evaluated.

N0: There is no evidence of cancer in the regional lymph nodes.

N1: Cancer has spread to lymph nodes above the triangular area described above. The lymph nodes are on the same side as the primary tumor, and the cancer found in the lymph nodes is 6 centimeters (cm) or smaller.

N2: Cancer has spread to lymph nodes on both sides of the neck, above the triangular area, but the cancer is 6 cm or smaller.

N3: Cancer found in lymph nodes is larger than 6 cm or is found in lymph nodes located in the triangle.

N3a: Cancer found in the lymph nodes is larger than 6 cm.

N3b: Cancer has extended to the triangle region.

Distant metastasis. The "M" in the TNM system indicates whether the cancer has spread to other parts of the body.

MX: Distant metastasis cannot be evaluated.

M0: Cancer has not spread to other parts of the body.

M1: Cancer has spread to other parts of the body.

Cancer stage grouping

Doctors assign the stage of the cancer by combining the T, N, and M classifications.

Stage 0: A carcinoma in situ (Tis) with no spread to lymph nodes (N0) or distant metastasis (M0).

Stage I: A small tumor (T1) with no spread to lymph nodes (N0) and no distant metastasis (M0).

Stage IIA: A tumor that has extended beyond the nasopharynx (T2) but has not spread to lymph nodes (N0) or to distant parts of the body (M0).

Stage IIB: A tumor (T1 or T2) that has spread to lymph nodes (N1) but has not metastasized (M0).

Stage III: This describes a noninvasive and invasive tumor (T1 or T2) that have spread to lymph nodes (N1 or N2) but have not metastasized (M0), or it describes a larger tumor (T3) with or without nodal involvement (N0, N1, or N2) and no metastasis (M0).

Stage IVA: This describes any invasive tumor (T4) with either no lymph node involvement (N0) or spread to only a single same-sided lymph node (N1) but no metastasis (M0). It is also used for any cancer (any T) with more significant nodal involvement (N2) but no metastasis (M0).

Stage IVB: This describes any tumor (any T) with extensive nodal involvement (N3a or N3b) but no metastasis (M0).

Stage IVC: This describes any tumor (any T, any N) when there is evidence of distant spread (M1).

Histologic grade (G). Doctors also describe a primary tumor by its grade, which is determined by using a microscope to examine tissue from a tumor (called a histologic examination). The doctor compares the tumor tissue with normal tissue. Histologic grade describes how closely the cancer cells resemble normal tissue under a microscope. Normal tissue contains many different types of cells grouped together, which is called differentiated. Tissue from a tumor usually has cells that look more alike each other (called poorly differentiated). Generally, the lower the grade, the better the prognosis. A tumor's grade is described using the letter "G" and a number.

GX: The grade cannot be evaluated.

G1: The cells look more like normal tissue (well differentiated).

G2: The cells are only moderately differentiated.

G3: The cells don’t resemble normal tissue (poorly differentiated).

Recurrent: Recurrent cancer is cancer that comes back after treatment. If there is a recurrence, the cancer may need to be staged again (called re-staging) using the system above.

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