Nasopharyngeal Cancer

Nasopharyngeal cancer is a rare type of cancer that starts in the upper part of the throat, behind the nose. While uncommon in most parts of the world, it occurs much more frequently in certain regions of Asia and Africa. Early symptoms can be hard to recognize, but understanding this disease and its treatment options can help patients and their families navigate the journey ahead.

Table of contents

• What is Nasopharyngeal Cancer?
• How Common is This Cancer?
• Signs and Symptoms
• Causes and Risk Factors
• Types of Nasopharyngeal Cancer
• Diagnosis and Testing
• Treatment Options
• Support and Coping

What is Nasopharyngeal Cancer?

Nasopharyngeal cancer is a disease in which cancer cells form in the tissues of the nasopharynx, which is the upper part of the throat behind the nose^[1]. The nasopharynx connects the back of your nose to the back of your mouth and sits just above the roof of your mouth, at the base of your skull^[2]. When you breathe in through your nose, air flows through your nose, nasopharynx, and into your throat before it reaches your lungs^[2].

The throat, also called the pharynx, is a muscular tube about 5 inches long that contains three sections. The nasopharynx is the topmost section, the oropharynx is the middle part located at the back of the mouth, and the hypopharynx is the lowermost part that opens to both the windpipe and food pipe^[6].

Nasopharyngeal cancer begins when cells in the nasopharynx start to grow out of control and form cancerous tumors. These tumors can spread to nearby lymph nodes in the neck, and in more advanced cases, to other parts of the body such as the liver, lungs, and bones^[2].

How Common is This Cancer?

Nasopharyngeal cancer is rare in the United States and other Western countries, affecting about 1 out of 100,000 people each year^[2]. In the United Kingdom, approximately 260 people are diagnosed with this cancer annually^[3]. However, it happens much more often in other parts of the world, mainly Southeast Asia, southern China, North Africa, and the Middle East^[1].

In areas where the disease is common, such as southern China, the incidence can be as high as 25 to 50 cases per 100,000 people per year^[4]. The disease is responsible for a complex interaction of genetic factors and virus exposure^[4].

About three times as many men as women are affected by nasopharyngeal cancer^[3]. In the United States, it typically affects people ages 15 to 24 and ages 65 to 79^[2]. In areas where the disease is not common, it is more likely to be diagnosed in people who are older than 50 years, but in high-risk areas, younger people are more likely to be affected^[6].

Signs and Symptoms

Nasopharyngeal cancer may not cause any symptoms at first. It is often difficult to recognize because the symptoms are similar to other, less serious conditions^[3]. Many people with nasopharyngeal cancer don’t have any symptoms until the cancer reaches an advanced stage^[3].

In most cases, people with nasopharyngeal cancer notice a lump on the back of their neck. There may be one or multiple lumps, and they’re usually not painful^[2]. These masses appear when cancer spreads to the lymph nodes in your neck and causes them to swell. Lymph nodes are small, bean-shaped glands that are part of the body’s defense system against infection^[2].

Other common warning signs include^[1][2][3]:

• Tinnitus, or ringing in your ears
• Hearing loss, usually only in one ear
• A feeling of fullness in your ears
• Ear infections that won’t go away
• Headaches
• Nasal stuffiness or a blocked nose, usually only on one side
• Nosebleeds
• Bleeding from the nose or bloody saliva
• Sore throat
• Facial pain or numbness
• Double vision or blurred vision
• Difficulty opening your mouth
• Difficulty breathing or speaking
• Difficulty swallowing
• A hoarse voice
• Unexplained weight loss

You should talk to a healthcare provider if you have any of these symptoms, particularly if they haven’t improved after 3 weeks or if they last for more than two weeks or keep coming back^[2][3]. Having one or more of these symptoms doesn’t mean you have nasopharyngeal cancer, but it’s best to get them checked out.

Causes and Risk Factors

The exact cause of nasopharyngeal cancer is unknown, but several factors can increase your risk of developing the condition^[3]. Nasopharyngeal cancer is caused by certain changes in how cells in the nasopharynx function, especially how they grow and divide into new cells^[6].

Important risk factors include^[3][6][11]:

• Being infected with Epstein-Barr virus (EBV), a common virus that causes glandular fever. EBV infection is strongly associated with nasopharyngeal cancer, especially in areas where the disease is common^[4]
• Living in or having ancestry in certain parts of Asia (especially southern China or Southeast Asia), North Africa, and the Arctic
• Being of south Chinese or north African descent
• Having a family member with nasopharyngeal cancer, particularly a first-degree relative such as a parent
• Using tobacco or breathing in secondhand smoke
• Frequent and heavy consumption of alcoholic beverages
• Having a diet very high in salt-cured meats and fish, which may contain cancer-causing chemicals such as nitrosamines^[3]
• Having a job where you regularly come into contact with hardwood dust or a chemical called formaldehyde

The human papilloma virus (HPV) is also thought to be a risk factor for developing nasopharyngeal cancer^[3].

Types of Nasopharyngeal Cancer

There are different kinds of tissue in your nasopharynx, and each kind contains different types of cells. Nasopharyngeal cancer subtypes are classified by the type of cell affected^[2]. According to the World Health Organization, nasopharyngeal cancer is classified into three main types^[2][4]:

• Keratinizing squamous cell carcinoma (WHO type 1): Cancer cells in the tissue lining your nasopharynx that are covered with keratin, a protein found in your hair and nails. This type accounts for 20 to 25 percent of cases and is the most common type in areas with low rates of nasopharyngeal cancer, such as the United States
• Non-keratinizing differentiated carcinoma (WHO type 2): Cancer cells in nasopharynx tissues that aren’t covered with keratin. This type accounts for 10 to 15 percent of cases
• Non-keratinizing undifferentiated carcinoma (WHO type 3): These are cancer cells that look very different from healthy cells when viewed under a microscope. This type accounts for 60 to 65 percent of cases. These cells grow and spread very quickly

The non-keratinizing types are most commonly associated with EBV infection and are found mostly in Asia^[4]. The treatment is generally the same for all types of nasopharyngeal cancer, but the stage of the cancer is often more important than the type in determining outlook and treatment options.

Diagnosis and Testing

Nasopharyngeal cancer diagnosis often begins with an exam by a healthcare professional who may ask about your symptoms and your habits^[13]. If your doctor thinks further tests are necessary, you will be referred to a head and neck cancer specialist^[3].

Several tests may be carried out to check for nasopharyngeal cancer and rule out other conditions^[3][13]:

Physical exam: A healthcare professional will do a physical exam to look for signs of cancer. This might include examining your throat using a small mirror and a light, and feeling your neck for swelling in the lymph nodes^[3][13].

Endoscopy: A procedure called a nasal endoscopy uses a thin, flexible tube with a tiny camera on the end to look inside your nasopharynx^[13]. The endoscope might go through your nose to see your nasopharynx, or through the opening in the back of your throat. You’re usually conscious during this procedure, but local anesthetic can be used to numb your nose and throat^[3].

Biopsy: A biopsy is a procedure to remove a sample of tissue for testing in a laboratory^[13]. A healthcare professional might take the sample during a nasal endoscopy procedure by putting special tools through the endoscope to remove some tissue. If there is swelling in the lymph nodes in the neck, a needle might be used to draw out some cells for testing.

Imaging scans: Once the diagnosis is confirmed, other tests can find the extent, called the stage, of the cancer. These might include^[3][13]:

• MRI scans (Magnetic Resonance Imaging)
• CT scans (Computed Tomography scans)
• PET-CT scans (Positron Emission Tomography-CT scans)
• X-rays

The stages of nasopharyngeal cancer range from 0 to 4. A lower stage number means the cancer is less advanced and has not spread far from where it started^[13].

Treatment Options

If you’re diagnosed with nasopharyngeal cancer, you’ll be cared for by a team of different specialists who work together^[3]. Members of your medical team will discuss with you what they think the best treatment option is in your case.

The two main treatments for nasopharyngeal cancer are^[3]:

Radiotherapy: Radiotherapy, also called radiation therapy, uses high-energy radiation beams to kill cancer cells^[3]. It is the most commonly used treatment for nasopharyngeal cancer. It can be used on its own to treat very early-stage cancers, or in combination with chemotherapy for more advanced cancers. Most of the time, external radiotherapy is used, where a machine focuses radiation beams on the area that needs treatment.

Chemotherapy: Chemotherapy uses medicine to kill cancer cells^[3]. It is usually given with radiation therapy (called chemoradiation) or it may be used alone.

A combination of radiotherapy and chemotherapy is often used^[3]. Surgery isn’t usually used to treat nasopharyngeal cancer because it’s difficult for surgeons to access the affected area^[3].

If you smoke, it’s important you give up. Smoking increases your risk of cancer returning and may cause more side effects from treatment^[3].

Support and Coping

Doctors, nurses, and other specialists collaborate to provide support for people with nasopharyngeal cancer throughout diagnosis and treatment and during follow-up appointments^[24].

The schedule for follow-up appointments tends to vary from person to person. Doctors may see you every month during the first year after treatment and then every 2 to 3 months during the second year. During the third year, they may see you every 4 to 6 months, and then every 6 to 12 months in the fourth and fifth years after treatment^[24].

During these appointments, your doctor may perform a physical exam, an endoscopy, and imaging tests. This helps the doctor monitor for any recurrence. Because radiation therapy can damage certain glands, your doctor may check the function of your thyroid and pituitary glands with a blood test during follow-up visits^[24].

Speech and swallowing therapy: Speech and swallowing therapists can meet with you before treatment to discuss how therapies for nasopharyngeal cancer may affect your ability to speak and swallow^[24]. Radiation therapy and chemoradiation can cause dry mouth and inflammation of the throat, making speech and swallowing difficult. Therapy may be used throughout treatment and during recovery to help you maintain or regain function.

Dental health: Your doctor may recommend that you see a dentist before, during, and after treatment, because radiation therapy for nasopharyngeal cancer can sometimes cause dry mouth, resulting in tooth decay^[24]. Regularly brushing and flossing your teeth, daily fluoride treatments, using mouthwash, and having frequent dental checkups can help prevent these problems.

Nutrition support: Because nasopharyngeal cancer and its treatments often cause swallowing problems, you may need a nutritional assessment and dietary plan^[24]. Nutritionists can help ensure you are getting the nutrients you need in easy-to-swallow foods throughout treatment and recovery.

Emotional support: You are likely to have a range of emotions that change very quickly. You might feel upset, frightened and confused^[20]. Counseling can help you cope with the difficulties you’ll face and reduce stress. Talking to your friends and relatives about your cancer can help and support you. You might also find it easier to talk to someone other than your own friends and family, such as a specialist nurse or a counselor.