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Tumors of many histologies can occur in the nasopharynx, but only nasopharyngeal carcinomas (also called NPC) are covered in this summary. The American Joint Committee on Cancer nasopharynx staging refers exclusively to the World Health Organization's (WHO) classification of grades I, II, and III nasopharyngeal carcinoma.
Incidence and Mortality
Less than one person out of 100,000 is diagnosed with nasopharyngeal carcinoma in the world each year, with most cases found in southern China, Southeast Asia, the Arctic, and the Middle East/North Africa. The incidence is higher in males than in females.[
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. Nasopharyngeal carcinoma originates from the epithelial cells that line the nasopharynx.
Anatomy of the pharynx.
Risk Factors
Risk factors for nasopharyngeal carcinoma include the following:[
Risk factors for keratinizing squamous cell carcinoma (WHO grade I):
Risk factors for nonkeratinizing carcinoma (WHO grades II and III):
Clinical Features
Signs and symptoms at presentation include the following:
In patients who present with cervical adenopathy alone, the finding of EBV genomic material in the tissue using polymerase chain reaction (PCR) is strong evidence of a nasopharyngeal primary tumor, and that area should be examined closely.[
Diagnostic Evaluation
Diagnostic tests and procedures
Diagnosis is made by biopsy of the nasopharyngeal mass. The following tests and procedures are used in the diagnosis of nasopharyngeal carcinoma:[
Any clinical or laboratory finding that suggests distant metastasis may prompt further evaluation of other sites. MRI is often more helpful than CT scans in assessing skull base involvement and in defining the extent of abnormalities detected.[
Circulating cancer-derived EBV DNA
EBV DNA in plasma samples in endemic populations may be useful in screening for early asymptomatic nasopharyngeal carcinoma. Circulating cancer-derived EBV DNA in plasma is an established tumor marker for nasopharyngeal carcinoma, with a sensitivity of 96% and a specificity of 93%.[
Evidence (EBV DNA in plasma for screening and diagnosis of nasopharyngeal carcinoma):
HPV
Differentiating HPV-related nasopharyngeal carcinoma requires identification of p16 immunohistochemical staining, in situ hybridization, and/or PCR similar to the method for differentiating HPV-related oropharyngeal cancer. Less than 10% of nonkeratinizing nasopharyngeal carcinomas are associated with HPV infection.[
Prognostic Factors
Major prognostic factors that adversely influence treatment outcome include the following:[
Follow-Up Testing and Late Effects
Follow-up testing for tumor recurrence includes the following:[
Patients should be monitored for the following potential late effects of treatment:[
Although most recurrences occur within 5 years of diagnosis, relapse can be seen at longer intervals. The incidence of second primary malignancies after treatment is lower for nasopharyngeal carcinoma than for other head and neck cancer sites.[
Accumulating evidence has demonstrated a high incidence (>30%–40%) of hypothyroidism in patients who have received radiation therapy that delivered external-beam radiation therapy (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.[
Careful dental and oral hygiene evaluation and therapy is particularly important before initiation of radiation treatment. 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 (2DRT).[
Evidence (IMRT vs. 2DRT and incidence of xerostomia):
References:
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 version.[
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.[
References:
Staging systems used for clinical staging are based on the best possible estimate of the extent of disease before treatment.[
Assessment of the primary tumor is made on the basis of inspection, 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 important for tumors of the nasopharynx. Nodal drainage areas are examined by careful palpation and radiological evaluation. The retropharyngeal lymph nodes are the first echelon of drainage.[
Information from the following diagnostic imaging studies may be used in staging:
If a patient has a relapse, a complete reassessment must be done to select the appropriate additional therapy.
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 carcinoma.[
Stage | TNM | Description |
---|---|---|
T = primary tumor; N = regional lymph node; M = distant metastasis. | ||
a Reprinted with permission from AJCC: Nasopharynx. In: Amin MB, Edge SB, Greene FL, et al., eds.:AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 103–11. | ||
0 | Tis, N0, M0 | Tis = Carcinomain situ. |
N0 = No regional lymph node metastasis. | ||
M0 = No distant metastasis. |
Stage | TNM | Description |
---|---|---|
T = primary tumor; N = regional lymph node; M = distant metastasis. | ||
a Reprinted with permission from AJCC: Nasopharynx. In: Amin MB, Edge SB, Greene FL, et al., eds.:AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 103–11. | ||
I | T1, N0, M0 | T1 = Tumor confined to nasopharynx, or extension to oropharynx and/or nasal cavity without parapharyngeal involvement. |
N0 = No regional lymph node metastasis. | ||
M0 = No distant metastasis. |
Stage | TNM | Description |
---|---|---|
T = primary tumor; N = regional lymph node; M = distant metastasis; EBV = Epstein-Barr virus. | ||
a Reprinted with permission from AJCC: Nasopharynx. In: Amin MB, Edge SB, Greene FL, et al., eds.:AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 103–11. | ||
II | T0, Tis, T1, N1, M0 | T0 = No tumor identified, but EBV-positive cervical node(s) involvement. |
Tis = Carcinomain situ. | ||
T1 = Tumor confined to nasopharynx, or extension to oropharynx and/or nasal cavity without parapharyngeal involvement. | ||
N1 = Unilateral metastasis in cervical lymph node(s) and/or unilateral or bilateral metastasis in retropharyngeal lymph node(s), ≤6 cm in greatest dimension, above the caudal border of cricoid cartilage. | ||
M0 = No distant metastasis. | ||
T2, N0, M0 | T2 = Tumor with extension to parapharyngeal space, and/or adjacent soft tissue involvement (medial pterygoid, lateral pterygoid, prevertebral muscles). | |
N0 = No regional lymph node metastasis. | ||
M0 = No distant metastasis. | ||
T2, N1, M0 | T2 = Tumor with extension to parapharyngeal space, and/or adjacent soft tissue involvement (medial pterygoid, lateral pterygoid, prevertebral muscles). | |
N1 = Unilateral metastasis in cervical lymph node(s) and/or unilateral or bilateral metastasis in retropharyngeal lymph node(s), ≤6 cm in greatest dimension, above the caudal border of cricoid cartilage. | ||
M0 = No distant metastasis. |
Stage | TNM | Description |
---|---|---|
T = primary tumor; N = regional lymph node; M = distant metastasis; EBV = Epstein-Barr virus. | ||
a Reprinted with permission from AJCC: Nasopharynx. In: Amin MB, Edge SB, Greene FL, et al., eds.:AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 103–11. | ||
III | T0, Tis, T1, N2, M0 | T0 = No tumor identified, but EBV-positive cervical node(s) involvement. |
Tis = Carcinomain situ. | ||
T1 = Tumor confined to nasopharynx, or extension to oropharynx and/or nasal cavity without parapharyngeal involvement. | ||
N2 = Bilateral metastasis in cervical lymph node(s), ≤6 cm in greatest dimension, above the caudal border of cricoid cartilage. | ||
M0 = No distant metastasis. | ||
T2, N2, M0 | T2 = Tumor with extension to parapharyngeal space, and/or adjacent soft tissue involvement (medial pterygoid, lateral pterygoid, prevertebral muscles). | |
N2 = Bilateral metastasis in cervical lymph node(s), ≤6 cm in greatest dimension, above the caudal border of cricoid cartilage. | ||
M0 = No distant metastasis. | ||
T3, N0, M0 | T3 = Tumor with infiltration of bony structures at skull base, cervical vertebra, pterygoid structures, and/or paranasal sinuses. | |
N0 = No regional lymph node metastasis. | ||
M0 = No distant metastasis. | ||
T3, N1, M0 | T3 = Tumor with infiltration of bony structures at skull base, cervical vertebra, pterygoid structures, and/or paranasal sinuses. | |
N1 = Unilateral metastasis in cervical lymph node(s) and/or unilateral or bilateral metastasis in retropharyngeal lymph node(s), ≤6 cm in greatest dimension, above the caudal border of cricoid cartilage. | ||
M0 = No distant metastasis. | ||
T3, N2, M0 | T3 = Tumor with infiltration of bony structures at skull base, cervical vertebra, pterygoid structures, and/or paranasal sinuses. | |
N2 = Bilateral metastasis in cervical lymph node(s), ≤6 cm in greatest dimension, above the caudal border of cricoid cartilage. | ||
M0 = No distant metastasis. |
Stage | TNM | Description |
---|---|---|
T = primary tumor; N = regional lymph node; M = distant metastasis; EBV = Epstein-Barr virus. | ||
a Reprinted with permission from AJCC: Nasopharynx. In: Amin MB, Edge SB, Greene FL, et al., eds.:AJCC Cancer Staging Manual. 8th ed. New York, NY: Springer, 2017, pp 103–11. | ||
IVA | T4, N0, M0 | T4 = Tumor with intracranial extension, involvement of cranial nerves, hypopharynx, orbit, parotid gland, and/or extensive soft tissue infiltration beyond the lateral surface of the lateral pterygoid muscle. |
N0 = No regional lymph node metastasis. | ||
M0 = No distant metastasis. | ||
T4, N1, M0 | T4 = Tumor with intracranial extension, involvement of cranial nerves, hypopharynx, orbit, parotid gland, and/or extensive soft tissue infiltration beyond the lateral surface of the lateral pterygoid muscle. | |
N1 = Unilateral metastasis in cervical lymph node(s) and/or unilateral or bilateral metastasis in retropharyngeal lymph node(s), ≤6 cm in greatest dimension, above the caudal border of cricoid cartilage. | ||
M0 = No distant metastasis. | ||
T4, N2, M0 | T4 = Tumor with intracranial extension, involvement of cranial nerves, hypopharynx, orbit, parotid gland, and/or extensive soft tissue infiltration beyond the lateral surface of the lateral pterygoid muscle. | |
N2 = Bilateral metastasis in cervical lymph node(s), ≤6 cm in greatest dimension, above the caudal border of cricoid cartilage. | ||
M0 = No distant metastasis. | ||
Any T, N3, M0 | TX = Primary tumor cannot be assessed. | |
T0 = No tumor identified, but EBV-positive cervical node(s) involvement. | ||
Tis = Carcinomain situ. | ||
T1 = Tumor confined to nasopharynx, or extension to oropharynx and/or nasal cavity without parapharyngeal involvement. | ||
T2 = Tumor with extension to parapharyngeal space, and/or adjacent soft tissue involvement (medial pterygoid, lateral pterygoid, prevertebral muscles). | ||
T3 = Tumor with infiltration of bony structures at skull base, cervical vertebra, pterygoid structures, and/or paranasal sinuses. | ||
T4 = Tumor with intracranial extension, involvement of cranial nerves, hypopharynx, orbit, parotid gland, and/or extensive soft tissue infiltration beyond the lateral surface of the lateral pterygoid muscle. | ||
N3 = Unilateral or bilateral metastasis in cervical lymph node(s), >6 cm in greatest dimension, and/or extension below the caudal border of cricoid cartilage. | ||
M0 = No distant metastasis. | ||
IVB | Any T, Any N, M1 | Any T = See Stage IVA above. |
NX = Regional lymph nodes cannot be assessed. | ||
N0 = No regional lymph node metastasis. | ||
N1 = Unilateral metastasis in cervical lymph node(s) and/or unilateral or bilateral metastasis in retropharyngeal lymph node(s), ≤6 cm in greatest dimension, above the caudal border of cricoid cartilage. | ||
N2 = Bilateral metastasis in cervical lymph node(s), ≤6 cm in greatest dimension, above the caudal border of cricoid cartilage. | ||
N3 = Unilateral or bilateral metastasis in cervical lymph node(s), >6 cm in greatest dimension, and/or extension below the caudal border of cricoid cartilage. | ||
M1 = Distant metastasis. |
References:
Stage | Treatment Options |
---|---|
Stage I nasopharyngeal carcinoma | Radiation therapy |
Stages II, III, and IV nasopharyngeal carcinoma | Radiation therapy |
Concurrent chemoradiation | |
Neoadjuvant chemotherapy and concurrent chemoradiation | |
Concurrent chemoradiation and adjuvant chemotherapy | |
Neoadjuvant chemotherapy followed by radiation therapy alone | |
Surgery | |
Chemotherapy(for patients with stage IVC disease) | |
Metastatic and recurrent nasopharyngeal carcinoma | Radiation therapy |
Surgery(for highly selected patients) | |
Chemotherapy/immunotherapy |
Fluorouracil Dosing
The DPYD gene encodes an enzyme that catabolizes pyrimidines and fluoropyrimidines, like capecitabine and fluorouracil. An estimated 1% to 2% of the population has germline pathogenic variants in DPYD, which lead to reduced DPD protein function and an accumulation of pyrimidines and fluoropyrimidines in the body.[
References:
Treatment Options for Stage I Nasopharyngeal Carcinoma
Treatment options for stage I nasopharyngeal carcinoma include the following:
Radiation therapy
High-dose radiation therapy with chemotherapy is the initial treatment of nasopharyngeal carcinoma.[
Most tumors are exclusively treated with external-beam radiation therapy. For some patients, 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.[
Current Clinical Trials
Use our
References:
Treatment Options for Stages II, III, and IV Nonmetastatic Nasopharyngeal Carcinoma
Treatment options for stages II, III, and IV nonmetastatic nasopharyngeal carcinoma include the following:
Radiation therapy
High-dose radiation therapy with chemotherapy is the initial treatment of nasopharyngeal carcinoma.[
Most tumors are exclusively treated with external-beam radiation therapy (EBRT). For some patients, 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.[
Evidence (radiation therapy):
This trial shows that radiation therapy alone could be used for limited-stage disease if the EBV titers (which are not usually tested in the United States) show fewer than 4,000 copies/mL. Radiation therapy alone was not previously considered a standard of care, but based on these results, patients with lower-volume disease and a low EBV titer may consider radiation therapy alone.
Chemoradiation therapy
Studies and meta-analyses investigating chemoradiation combinations have been reported.[
Evidence (neoadjuvant chemotherapy vs. chemoradiation therapy):
Data from phase III randomized trials support induction chemotherapy with gemcitabine plus cisplatin before concurrent chemoradiation therapy.[
In a multicenter phase III trial, patients were randomly assigned to receive either concurrent chemoradiation therapy alone (standard therapy, n = 238) or gemcitabine and cisplatin induction chemotherapy before concurrent chemoradiation therapy (n = 242). With a median follow-up of 69.8 months, patients in the induction chemotherapy group had a significantly higher 5-year OS rate (87.9%) than those in the standard therapy group (78.8%) (HR, 0.51; 95% CI, 0.34–0.78; P = .001). The risk of late toxicities was comparable (grade 3 or higher toxicity, 11.3% vs. 11.4%).[
Evidence (chemoradiation therapy plus adjuvant chemotherapy):
Evidence (combination chemotherapy plus radiation therapy vs. radiation therapy alone):
Evidence (chemoradiation therapy using carboplatin vs. cisplatin):
Surgery
Neck dissection may be indicated for patients with persistent or recurrent lymph nodes if the primary tumor site is controlled.[
Chemotherapy
Chemotherapy is given to patients with stage IVC disease.[
Clinical trials for patients with 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.[
Current Clinical Trials
Use our
References:
Treatment Options for Metastatic and Recurrent Nasopharyngeal Carcinoma
Treatment options for metastatic and recurrent nasopharyngeal carcinoma include the following:
Radiation therapy
High-dose radiation therapy with chemotherapy is the initial treatment of patients with nasopharyngeal carcinoma for the primary tumor site and the neck.[
Most tumors are treated with EBRT exclusively. For some patients, 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.[
Surgery
In highly selected patients, surgical resection of locally recurrent lesions may be considered.
Chemotherapy/Immunotherapy
If a patient has metastatic disease or local recurrence that is no longer amenable to surgery or radiation therapy, chemotherapy or immunotherapy may be considered.[
Evidence (chemotherapy/immunotherapy):
The U.S. Food and Drug Administration has approved toripalimab with cisplatin and gemcitabine as first-line treatment for patients with metastatic or recurrent locally advanced nasopharyngeal carcinoma. It is also approved as a single agent for adults with recurrent unresectable or metastatic nasopharyngeal carcinoma with disease progression during or after platinum-containing therapy.
Current Clinical Trials
Use our
References:
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.
Treatment Option Overview for Nasopharyngeal Carcinoma
Revised Table 6, Treatment Options for Nasopharyngeal Carcinoma.
Treatment of Metastatic and Recurrent Nasopharyngeal Carcinoma
The Chemotherapy/Immunotherapy subsection was renamed from Chemotherapy and extensively revised.
This summary is written and maintained by the
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 carcinoma. It is intended as a resource to inform and assist clinicians in the care of their 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
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 Carcinoma Treatment are:
Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's
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 Carcinoma Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at:
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Last Revised: 2024-07-25
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