What are the other Names for this Condition? (Also known as/Synonyms)

• Canton Tumor
• Carcinoma of Nasopharynx

What is Nasopharyngeal Carcinoma? (Definition/Background Information)

• Nasopharyngeal Carcinoma is a malignant tumor of the back of the nose; in the region, where the top of the throat meets the back of the nose. This region is called the “nasopharynx”
• The tumor arises from this location and starts invading surrounding areas. In particular, the tumor growth starts in the epithelium (outer lining) of an important spot in the nasopharynx called the “Fossa of Rossenmuller”, a common site of origin for Nasopharyngeal Carcinomas
• Many Nasopharyngeal Carcinoma tumors are observed in middle-aged adult males. The cause of formation of the tumor is generally unknown, but it seems to be associated with Epstein-Barr virus infection, for some unknown reason
• The signs and symptoms of Nasopharyngeal Carcinoma may include the presence of a tumor mass in the nasopharyngeal region, obstruction of the nasal sinuses, headaches, blood in sputum or nasal discharge, and hearing and swallowing difficulties
• The treatment for Nasopharyngeal Carcinoma involves a combination of chemotherapy, radiation therapy, and in some cases, surgery. With prompt diagnosis and adequate treatment, the prognosis is generally good

Who gets Nasopharyngeal Carcinoma? (Age and Sex Distribution)

• Individuals between the ages of 50 and 59 years have the highest incidence rate for Nasopharyngeal Carcinoma. However, once an individual reaches the age of 59 years, the risk of developing this malignant tumor begins to decrease
• Men are two times more likely to develop Nasopharyngeal Carcinoma than women
• All races and ethnic groups are prone to this malignancy
• This type of cancer is a rare within the United States. However, in other parts of the world, such as Southeast Asia, it is observed at a higher frequency rate

What are the Risk Factors for Nasopharyngeal Carcinoma? (Predisposing Factors)

Researchers have identified certain factors that increase an individual’s risk for developing Nasopharyngeal Carcinoma, and these include:

• Nasopharyngeal Carcinoma most commonly affects individuals within Asia and Northern Africa. In addition, Asians who immigrate to the United States have an increased risk of developing this type of cancer than do American-born Asians
• Inuits, a group of indigenous peoples that inhabit the Arctic regions of Greenland, Canada, and United States, are also known to have a higher risk of developing this type of cancer, than the general population
• Nasopharyngeal Carcinoma can occur at any age, but it is usually diagnosed in adults between 30 and 50 years old
• Frequent consumption of certain types of salt-cured foods high in butyric acid (such as fish and preserved vegetables) increases the risk
• High nitrosamine food diet
• Individuals with a family history of Nasopharyngeal Carcinoma
• Excessive alcohol consumption
• Nasopharyngeal Carcinoma is reportedly linked to Epstein-Barr virus (EBV) infection

It is important to note that having a risk factor does not mean that one will get the condition, A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Nasopharyngeal Carcinoma? (Etiology)

The exact cause of Nasopharyngeal Carcinoma is not completely understood.

• However, it is observed that there is a strong correlation between Nasopharyngeal Carcinoma and being infected with the Epstein-Barr virus, the virus causing mononucleosis infection
• But, not all individuals with EBV infection develop Nasopharyngeal Carcinoma

What are the Signs and Symptoms of Nasopharyngeal Carcinoma?

During the early-stages of Nasopharyngeal Carcinoma, significant signs and symptoms may not be observed. However, the signs and symptoms that may occur during the later stages of tumor growth and development include:

• Headache (cephalgia)
• Presence of a non-painful lump in the neck; foreign body sensation in the throat
• Nasal bleeding and discharge; nasal blockage/ congestion
• Blood in saliva
• Loss of hearing
• Recurring ear infections
• Difficulty swallowing
• Change in voice

How is Nasopharyngeal Carcinoma Diagnosed?

Diagnostic methods used to diagnose Nasopharyngeal Carcinoma may include:

• Diagnosing Nasopharyngeal Carcinoma usually starts with a thorough physical examination and complete medical history. The healthcare provider usually applies hand pressure to the neck, to feel if a lump or bulge is present in the nasopharyngeal region
• Complete blood count (CBC) to evaluate red and white blood cells and platelet counts
• An MRI scan of the head and neck area is used to provide a clear image of any soft tissue invasion
• Positron emission tomography (PET) scan: In certain cases, a healthcare provider may order a PET scan, to see if the tumor has any malignant characteristics
• Epstein Barr virus (EBV) blood test may be performed, to test for antibodies and DNA markers of EBV
• Hearing test: The tumor may affect the ability to hear properly. Hence, this procedure is performed to test the hearing capacity of an individual, such as if soft/loud sounds and low/high pitched sounds are heard. Each ear is tested independently
• Histopathological studies conducted on a biopsy specimen - the specimen is examined under a microscope by a pathologist, to arrive at a definitive diagnosis Once the diagnosis is confirmed, the healthcare provider may order a CT scan in certain cases to see the size of the tumor, the direction in which it is spreading, and if the skull or other regions are affected

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Nasopharyngeal Carcinoma?

The complications of Nasopharyngeal Carcinoma may include:

• Severe emotional distress due to the presence of cancer
• Nasopharyngeal Carcinoma is a highly-malignant tumor that tends to spread early (metastatic Nasopharyngeal Carcinoma). Usually, by the time it is diagnosed, there is a 90% chance that the tumor has already spread to the head and neck region. There is also a 10% chance that the tumor has spread to other parts of the body, such as to the lungs or liver
• Obstruction of the sinus orifice can cause severe sinusitis (sinus infection)
• Due to the metastasizing or malignant nature of Nasopharyngeal Carcinoma, it can cause frequent nosebleeds. The bleeding can be frequent and severe, which can result in anemia
• Side effects of radiation therapy and chemotherapy (such as toxicity)

How is Nasopharyngeal Carcinoma Treated?

The treatment methods for Nasopharyngeal Carcinoma are determined by several factors, such as:

• The type of cancer
• How advanced the condition is
• The overall health of the affected individual
• As well as, his/her personal preferences

Determination of type and extent of cancer: Once a diagnosis of Nasopharyngeal Carcinoma has been established, the extent to which the tumor has spread is assessed, known as staging. The system used most often to stage Nasopharyngeal Carcinoma is the American Joint Committee on Cancer (AJCC) TNM system, which is based on 3 key information parameters.

The TNM classification for Nasopharyngeal Cancer is given below:

T groups for Nasopharyngeal Cancer:

• TX: Primary tumor cannot be assessed because of incomplete information
• T0: No evidence of a primary tumor
• Tis: Carcinoma in situ (cancer cells are found only in the surface layer of the nasopharynx, but have not invaded into deeper layers)
• T1: Tumor is in the nasopharynx. The cancer may also have grown into the oropharynx (the part of the throat in the back of the mouth) and/or nasal cavity, but no farther
• T2: The cancer has grown into the tissues of left or right sides of the upper part of the throat (but not into the bone)
• T3: The tumor has grown into the sinuses and/or the bones nearby
• T4: The tumor has grown into the skull and/or cranial nerves (nerves in the head that lie near the nasopharynx and have special functions such as vision, smell, and eye movement), the hypopharynx (lower part of the throat), or the eye or its nearby tissues

N groups for Nasopharyngeal Cancer:

• NX: Nearby lymph nodes cannot be assessed due to incomplete information
• N0: No spread to nearby lymph nodes
• N1: Spread to 1 or more lymph nodes on one side of the neck; OR, spread to lymph nodes behind the throat (called retropharyngeal lymph nodes) on either side of the neck. In either case, no lymph node is larger than 6 cm (about 2½ inches) across
• N2: Spread to lymph nodes on both sides of the neck, none larger than 6 cm across
• N3: Spread to lymph nodes that are either:
  • N3a: Larger than 6 cm across
  • N3b: Located in the shoulder area just above the collarbone (this area is called the supraclavicular fossa)

M groups for Nasopharyngeal Cancer:

• M0: The cancer has not spread to tissues or organs far away from the nasopharynx
• M1: The cancer has spread to tissues or organs far away from the nasopharynx

Stage grouping: Once the T, N, and M categories have been assigned, this information is combined to assign an overall stage in a process called stage grouping. The stages identify tumors that have a similar outlook and are treated in a similar way.

Stage 0 (Tis, N0, M0):

• The cancer is “in situ”. Cancer cells are only in the surface layer of the nasopharynx, but have not yet grown into deeper layers (Tis)
• The cancer has not spread to nearby lymph nodes (N0) or distant sites (M0)

Stage I (T1, N0, M0):

• The tumor is in the nasopharynx and may have spread to soft tissues of the nasal cavity, and/or to the oropharynx (T1)
• It has not spread to nearby lymph nodes (N0) or distant sites (M0)

Stage II - either of the following:

• T2, N0, M0: The tumor has grown into the tissues of the left or right sides of the upper part of the throat (T2). It has not spread to nearby lymph nodes (N0) or distant sites (M0)

OR

• T1 or T2, N1, M0:
  • The tumor may still be confined to the nasopharynx, or it may have extended to the soft tissues of the nasal cavity or the oropharynx (T1), or the left or right sides of the upper part of the throat (T2)
  • It has spread to one or more nearby lymph nodes, none of which is larger than 6 cm (about 2½ inches) across
  • These lymph nodes are either neck lymph nodes on one side, or lymph nodes behind the throat (retropharyngeal lymph nodes) on either side (N1)
  • The cancer has not spread to distant sites (M0)

Stage III - either of the following:

• T3, N0 to N2, M0:
  • The tumor has spread to the sinuses, or the bones near the nasopharynx (T3)
  • It may or may not have spread to lymph nodes in the neck or behind the throat, but none of these lymph nodes is larger than 6 cm across (N0 to N2)
  • It has not spread to distant sites (M0)

OR

• T1 or T2, N2, M0:
  • The tumor may still be confined to the nasopharynx, or it may have grown into the soft tissues of the nasal cavity or the oropharynx (T1), or the left or right sides of the upper part of the throat (T2)
  • The tumor has spread into nearby neck lymph nodes on both sides, but none of the lymph nodes is larger than 6 cm across (N2)
  • The cancer has not spread to distant sites (M0)

Stage IVA (T4, N0 to N2, M0):

• The tumor has grown into the skull and/or cranial nerves, the hypopharynx (lower part of the throat), the eye, or its nearby tissues (T4)
• It may or may not have spread to nearby lymph nodes in the neck, none of which is larger than 6 cm across (N0 to N2)
• It has not spread to distant sites (M0)

Stage IVB (Any T, N3, M0):

• The tumor may or may not have extended into nearby soft tissues or bones (any T)
• It has spread to lymph nodes that are larger than 6 cm across and/or are located in the shoulder area above the collarbone (N3)
• The cancer has not spread to distant sites (M0)

Stage IVC (Any T, any N, M1):

• The tumor may or may not have extended into nearby soft tissues or bones (any T)
• It may or may not have spread to nearby lymph nodes (any N)
• It has spread to distant sites (M1)

Following is the staging protocol for Nasopharyngeal Carcinoma, according to the American Joint Committee on Cancer (AJCC), updated June 2016.

Once a definitive diagnosis of Nasopharyngeal Carcinoma has been made, staging is performed to determine how far the cancer has spread. The stage can describe:

• How many lymph nodes are affected (if any)
• Their locations in the body
• And, if other organs are being affected (metastatic Nasopharyngeal Carcinoma)

Staging is important because different treatment regimens are necessary, depending on the progression of the carcinoma to higher stages.

• Stage 0: A carcinoma in situ (CIS) with no spread to lymph nodes or distant metastasis. In CIS, the carcinoma is confirmed to the epithelium
• Stage I: A small invasive tumor that has not spread to lymph nodes and no distant metastasis.
• Stage IIA: A tumor that has extended past the nasopharynx but has not spread to lymph nodes or other parts of the body
• Stage IIB: A tumor that has spread to the lymph nodes, but has not metastasized to other organs
• Stage III: Is a tumor that can be noninvasive or invasive that has spread to the lymph nodes but has not metastasized. This stage can also be described as a larger tumor with or without lymph node involvement and no metastasis
• Stage IVA: This stage describes any invasive tumor that has no lymph node involvement or has only spread to a single same-sided lymph node with no metastasis. It is also used for any cancer that has significantly more lymph node involvement but no metastasis
• Stage IVB: This describes any tumor with widespread lymph node involvement, but has not spread to other body parts
• Stage IVC: This describes any tumor in which there is evidence that the cancer has spread to other body parts
• Recurrent: Recurrent cancer is cancer that has come back after treatment. If there is a recurrence, the cancer may need to be staged again. The process is called re-staging and uses the same system

A combination of treatments is used to effectively treat this cancer. The aggressiveness of the treatment is determined by disease/cancer progression (or stage of the tumor).

Surgery for Nasopharyngeal Carcinoma:

• A surgery may be undertaken to excise the tumor (if possible)
• Else, surgery to debulk the tumor prior to radiation therapy and/or chemotherapy, may be considered

Radiation therapy for Nasopharyngeal Carcinoma:

• This treatment is the main choice of treatment for stage I Nasopharyngeal Carcinoma
• Radiation therapy attempts to destroy cancer cells, by aiming high-energy beams at the cancer cells
• Radiation therapy is usually administered through a machine placed outside the body, called external beam radiation
• The side effects of radiation therapy include nausea, vomiting, fatigue, pain, risk of (a different type of) cancer later in life, and the risk of heart disease
• Radiation can damage healthy cells in addition to cancer cells, causing further complications
• Another type of radiation therapy is sometimes used in recurrent cases. It is administered internally, by a device positioned directly at, or close to the malignant tumor, called brachytherapy

Chemotherapy for Nasopharyngeal Carcinoma:

• This approach uses a combination of drugs to kill the cancerous cells and is most commonly used for Nasopharyngeal Carcinoma that has spread beyond the head and neck region
• The drugs may be administered intravenously (through a vein in the arm), orally (in a pill-form), or both. Drugs inserted directly into the bloodstream, travel through the body and kill the cancer cells
• There are three methods by which chemotherapy is used to treat Nasopharyngeal Carcinoma. And, these include:
  • Chemotherapy in addition to radiation therapy: When these two treatment methods are combined, chemotherapy enhances the effectiveness of radiation therapy. This combined treatment is called concomitant therapy or chemoradiation. However, side effects of chemotherapy get added to the side effects of radiation therapy, making concomitant therapy more difficult to tolerate for the individual
  • Chemotherapy after radiation therapy: The physician may recommend chemotherapy after radiation therapy or after concomitant therapy. However, there is some debate on whether additional chemotherapy improves the survival rate. Many individuals who undergo chemotherapy after concomitant therapy have difficulty in tolerating the side effects and are forced to stop treatment
  • Chemotherapy before radiation therapy: Chemotherapy that is administered before radiation therapy or before concomitant therapy is called neoadjuvant chemotherapy. However, additional research is needed to determine if neoadjuvant chemotherapy can improve the survival rate
• There can be severe side effects of chemotherapy, which includes fatigue, nausea, hair loss, anemia, elevated risk of infection, and drug-specific reactions.

How can Nasopharyngeal Carcinoma be Prevented?

Current medical research has not established a definitive method of preventing Nasopharyngeal Carcinoma. However, certain steps may be taken to help reduce the risk and these include:

• Maintaining good oral hygiene, such as brushing teeth twice daily and flossing
• Reducing the consumption of foods high in salt
• Avoiding alcohol and tobacco products
• Regular or annual physical checkups

What is the Prognosis of Nasopharyngeal Carcinoma? (Outcomes/Resolutions)

• The prognosis of Nasopharyngeal Carcinoma depends upon a set of several factors, which include:
  • Stage of tumor: With lower-stage tumors, when the tumor is confined to site of origin, the prognosis is usually excellent with appropriate therapy. In higher-stage tumors, such as tumors with metastasis, the prognosis is typically poor
  • The surgical resectability of the tumor (meaning, if the tumor can be removed completely)
  • Overall health of the individual: Individuals with overall excellent health have better prognosis compared to those with poor health
  • Age of the individual: Older individuals generally have poorer prognosis than younger individuals
  • Whether the tumor is occurring for the first time, or is a recurrent tumor. Recurring tumors have worse prognosis compared to tumors that do not recur
  • Response to treatment: Tumors that respond to treatment have better prognosis than tumors that do not respond to treatment
• Almost all individuals diagnosed and treated for stage 0 cancer survive for 5 years, post-diagnosis (5-year survival rate is 90%). The survival rate steadily drops with cancer stage progression, and the intermediate stage 5-year survival rate is 75%
• In individuals with advanced cases of Nasopharyngeal Carcinoma, the 5-year survival rate is less than 55% following diagnosis and treatment

Additional and Relevant Useful Information for Nasopharyngeal Carcinoma:

The following article link will help you understand other cancers and benign tumors:

http://www.dovemed.com/diseases-conditions/cancer/