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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.
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:
Signs and Symptoms
Symptoms and signs at presentation include the following:
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.
Diagnosis is made by biopsy of the nasopharyngeal mass. Workup includes the following:
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.[5,9,10]
EBV-DNA in plasma samples in endemic populations may be useful in screening for early asymptomatic nasopharyngeal cancer. In a study of 20,174 participants in China, 309 patients (1.5% of all participants, and 27.8% of those who initially tested positive) had persistently detectable EBV-DNA in plasma at baseline and follow up. Among the 309 participants, nasopharyngeal cancer was confirmed after nasal endoscopic examination, MRI, and biopsy in 34 patients (11.0%).
Major prognostic factors adversely influencing outcome of treatment include the following:
Other factors linked to diminished survival that were present in some, but not all, studies include the following:
Follow-up for patients includes the following:
Monitoring of patients should include the following:
Although most recurrences occur within 5 years of diagnosis, relapse can be seen at longer intervals. The incidences of second primary malignancies are fewer than after treatment of tumors at other head and neck sites.
Circulating cancer-derived EBV-DNA in plasma is an established tumor marker for nasopharyngeal cancer, with a sensitivity of 96% and a specificity of 93%.[6,7] The presence of short EBV-DNA fragments of fewer than 181 base pairs in the plasma of nasopharyngeal carcinoma patients suggests that EBV-DNA molecules are released into the circulation by apoptosis of cancer cells rather than by active viral replication.
There are some nonkeratinizing nasopharyngeal carcinomas that are associated with HPV infection. Differentiating HPV-related nasopharyngeal cancer requires identification of p16 immunohistochemical staining, in situ hybridization, and/or polymerase chain reaction in a similar way to how HPV-related oropharyngeal cancer is differentiated.
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.
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.
The World Health Organization (WHO) definition of nasopharyngeal carcinoma is a "carcinoma arising in the nasopharyngeal mucosa that shows light microscopic or ultrastructural evidence of squamous differentiation." The WHO classification for nasopharyngeal carcinoma has evolved over time and the 2005 classification is the current one. The three versions below are all used, and in particular, the undifferentiated carcinomas that carry the worst prognosis and the greatest sensitivity to chemoradiation are generally classified according to the 1978 definitions.
1978 WHO classification:
1991 WHO classification:
2005 WHO classification:
Previous subdivisions of nasopharyngeal carcinoma included lymphoepithelioma, which is now classified as WHO grade III and characterized by lymphoid infiltrate.
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 (1978) represent the endemic form of nasopharyngeal carcinoma and is found in Southern China.
The presence of keratin has been associated with reduced local control and survival.
Staging systems are for 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 (CT) scanning in the evaluation of skull base invasion and intracranial spread. Positron emission tomography scans combined with CT are helpful in radiation treatment planning for targeted delineation of the primary tumor and aid in the detection of metastatic nodal involvement and metastatic spread, such as that found in lung or skeletal metastases in patients with advanced nasopharyngeal cancer.
If a patient has a relapse, a complete reassessment must be done to select the appropriate additional therapy.
Epithelial tumors of the nasopharynx are staged using this staging system.
American Joint Committee on Cancer (AJCC) Stage Groupings and TNM Definitions
The AJCC has designated staging by TNM (tumor, node, metastasis) classification to define nasopharyngeal cancer.
Standard treatments for patients with nasopharyngeal cancer include the following:
High-dose radiation therapy with chemotherapy is the primary treatment of nasopharyngeal cancer for the primary tumor site and the neck. 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 Radiation Therapy Oncology Group (RTOG) study (RTOG-0225) showed the feasibility of IMRT in a multi-institutional setting and minimal grade 3 and 4 xerostomia rates. 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.[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 before therapy and as part of posttreatment follow-up.[14,15]
Treatments under clinical evaluation for patients with nasopharyngeal cancer include the following:
Information about ongoing clinical trials is available from the NCI website.
Standard Treatment Options for Stage I Nasopharyngeal Cancer
Standard treatment options for stage I nasopharyngeal cancer:
Current Clinical Trials
Use our advanced clinical trial search to find NCI-supported cancer clinical trials that are now enrolling patients. The search can be narrowed by location of the trial, type of treatment, name of the drug, and other criteria. General information about clinical trials is also available.
Standard Treatment Options for Stage II Nasopharyngeal Cancer
Standard treatment options for stage II nasopharyngeal cancer:
Standard Treatment Options for Stage III Nasopharyngeal Cancer
Standard treatment options for stage III nasopharyngeal cancer:
Treatment Options Under Clinical Evaluation for Stage III Nasopharyngeal Cancer
Treatment options under clinical evaluation for stage III nasopharyngeal cancer:
Two randomized, prospective trials compared combination chemotherapy (i.e., cisplatin, epirubicin, and bleomycin or cisplatin plus fluorouracil infusion) plus radiation therapy with radiation therapy alone.[Level of evidence: 1iiA];[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 concurrently given.
Clinical trials for advanced tumors evaluating the use of chemotherapy before radiation therapy, concurrent with radiation therapy, or as adjuvant therapy after radiation therapy should be considered.[20,21,22,23]
Standard Treatment Options for Stage IV Nasopharyngeal Cancer
Standard treatment options for stage IV nasopharyngeal cancer:
Treatment Options Under Clinical Evaluation for Stage IV Nasopharyngeal Cancer
Treatment options under clinical evaluation for stage IV nasopharyngeal cancer:
Clinical trials for advanced tumors to evaluate the use of chemotherapy before radiation therapy, concurrent with radiation therapy, or as adjuvant therapy after radiation therapy should be considered.[20,21,22,23]
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 (OS) for neoadjuvant docetaxel was 94.1% compared with the control arm, which was 67.7% (HR, 0.24; 95% CI, 0.078–0.73; P = .012).[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 with radiation therapy alone.[Level of evidence: 1iiA]; [25,26][Level of evidence: 1iiDii] Although disease-free survival (DFS) was improved in the chemotherapy group for both groups, improvement in OS was reported only from the Intergroup trial in which chemotherapy with cisplatin was concurrently given.
Standard Treatment Options for Recurrent Nasopharyngeal Cancer
Standard treatment options for recurrent nasopharyngeal cancer:
Treatment Options Under Clinical Evaluation for Recurrent Nasopharyngeal Cancer
Treatment options under clinical evaluation for recurrent nasopharyngeal cancer:
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Stage III Nasopharyngeal Cancer
Added Zhang et al. as reference 15.
Stage IV Nasopharyngeal Cancer
This summary is written and maintained by the PDQ Adult Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® - NCI's Comprehensive Cancer Database pages.
Purpose of This Summary
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of adult nasopharyngeal cancer. It is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.
Reviewers and Updates
This summary is reviewed regularly and updated as necessary by the PDQ Adult Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).
Board members review recently published articles each month to determine whether an article should:
Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.
The lead reviewers for Nasopharyngeal Cancer Treatment (Adult) are:
Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.
Levels of Evidence
Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Adult Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.
Permission to Use This Summary
PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as "NCI's PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary]."
The preferred citation for this PDQ summary is:
PDQ® Adult Treatment Editorial Board. PDQ Nasopharyngeal Cancer Treatment (Adult). Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/head-and-neck/hp/adult/nasopharyngeal-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389193]
Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in Visuals Online, a collection of over 2,000 scientific images.
Based on the strength of the available evidence, treatment options may be described as either "standard" or "under clinical evaluation." These classifications should not be used as a basis for insurance reimbursement determinations. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.
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Last Revised: 2019-08-30
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