History

Nasopharyngeal carcinoma rarely comes to medical attention before it has spread to regional lymph nodes. Enlargement and extension of the tumor in the nasopharynx may result in symptoms of nasal obstruction (eg, congestion, nasal discharge, bleeding), changes in hearing (usually associated with blockage of the eustachian tube, but direct extension into the ear is possible), and cranial nerve palsies (usually associated with extension of the tumor into the base of the skull). Approximately 15% of patients with nasopharyngeal carcinoma present with distant metastases.^[14]

One study indicated the following symptoms^[1]:

Nasal symptoms, including bleeding, obstruction, and discharge (78%)
Ear symptoms, including infection, deafness, and tinnitus (73%)
Headaches (61%)
Neck swelling (63%)

Physical Examination

The most common physical finding is a neck mass consisting of painless firm lymph node enlargement (80%).

Neck involvement is often bilateral; the most common nodes involved are the jugulodigastric, and upper and middle jugular nodes in the anterior cervical chain.

Cranial nerve palsy at initial presentation is observed in 25% of patients.

On nasopharyngoscopy, a mass arising in the nasopharynx is often visible. The most frequent site is the fossa of Rosenmüller.

A paraneoplastic osteoarthropathy has been described in patients with widespread metastatic or recurrent disease.

Differential Diagnoses

Sham JS, Poon YF, Wei WI, Choy D. Nasopharyngeal carcinoma in young patients. Cancer. 1990 Jun 1. 65(11):2606-10. [QxMD MEDLINE Link].
Pathmanathan R, Prasad U, Sadler R, Flynn K, Raab-Traub N. Clonal proliferations of cells infected with Epstein-Barr virus in preinvasive lesions related to nasopharyngeal carcinoma. N Engl J Med. 1995 Sep 14. 333(11):693-8. [QxMD MEDLINE Link].
Ung A, Chen CJ, Levine PH, Cheng YJ, Brinton LA, Chen IH, et al. Familial and sporadic cases of nasopharyngeal carcinoma in Taiwan. Anticancer Res. 1999 Jan-Feb. 19(1B):661-5. [QxMD MEDLINE Link].
Lu CC, Chen JC, Tsai ST, Jin YT, Tsai JC, Chan SH, et al. Nasopharyngeal carcinoma-susceptibility locus is localized to a 132 kb segment containing HLA-A using high-resolution microsatellite mapping. Int J Cancer. 2005 Jul 10. 115(5):742-6. [QxMD MEDLINE Link].
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Berry MP, Smith CR, Brown TC, Jenkin RD, Rider WD. Nasopharyngeal carcinoma in the young. Int J Radiat Oncol Biol Phys. 1980 Apr. 6(4):415-21. [QxMD MEDLINE Link].
Al-Sarraf M, LeBlanc M, Giri PG, et al. Chemoradiotherapy versus radiotherapy in patients with advanced nasopharyngeal cancer: phase III randomized Intergroup study 0099. J Clin Oncol. 1998 Apr. 16(4):1310-1317. [QxMD MEDLINE Link].
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Mertens R, Granzen B, Lassay L, Gademann G, Hess CF, Heimann G. Nasopharyngeal carcinoma in childhood and adolescence: concept and preliminary results of the cooperative GPOH study NPC-91. Gesellschaft für Pädiatrische Onkologie und Hämatologie. Cancer. 1997 Sep 1. 80(5):951-9. [QxMD MEDLINE Link].
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Kwong DL, Wei WI, Sham JS, et al. Sensorineural hearing loss in patients treated for nasopharyngeal carcinoma: a prospective study of the effect of radiation and cisplatin treatment. Int J Radiat Oncol Biol Phys. 1996 Sep 1. 36(2):281-9. [QxMD MEDLINE Link].
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Gastpar H, Wilmes E, Wolf H. Epidemiologic, etiologic and immunologic aspects of nasopharyngeal carcinoma [NPC]. J Med. 1981. DA - 19820212(4):257-84. [QxMD MEDLINE Link].
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Ayan I, Altun M. Nasopharyngeal carcinoma in children: retrospective review of 50 patients. Int J Radiat Oncol Biol Phys. 1996 Jun 1. 35(3):485-92. [QxMD MEDLINE Link].
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Media Gallery

MRI of the head and neck in a patient with nasopharyngeal carcinoma showing the primary tumor and cervical lymph node metastases
Intensity modulated radiotherapy images for a patient with nasopharyngeal carcinoma

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Table 1. AJCC Staging for Nasopharyngeal Cancer

Stage	T	N	M
0	Tis	No	M0
I	T1	N0	M0
II	T1	N1	M0
	T2	N0	M0
	T2	N1	M0
III	T1	N2	M0
	T2	N2	M0
	T3	N0	M0
	T3	N1	M0
	T3	N2	M0
IVA	T4	N0	M0
	T4	N1	M0
	T4	N2	M0
IVB	Any T	N3	M0
IVC	Any T	Any N	MI

Table 2. Tumor (T) Staging

TX	Primary tumor cannot be assessed
T0	No evidence of primary tumor
Tis	Carcinoma in situ
T1	Tumor confined to the nasopharynx or extends to oropharynx and/or nasal cavity without parapharyngeal extension
T2	Tumor with parapharyngeal extension
T3	Tumor involves bony structures of skull base and/or paranasal sinuses
T4	Tumor with intracranial extension and/or involvement of cranial nerves, hypopharynx, orbit, or with extension to the infratemporal fossa/masticator space

Table 3. Nodal (N) Staging

NX	Regional lymph nodes cannot be assessed
N0	No regional lymph node metastasis
N1	Unilateral metastasis in cervical lymph node(s), less than or equal to 6 cm in greatest dimension, above the supraclavicular fossa, and/or unilateral or bilateral retropharyngeal lymph nodes, less than or equal to 6 cm in greatest dimension
N2	Bilateral metastasis in a cervical lymph node (s), less than or equal to 6 cm in greatest dimension, above the supraclavicular fossa
N3	Metastasis in a lymph node(s) greater than 6 cm and/or to supraclavicular fossa
N3a	Greater than 6 cm in dimension
N3b	Extension to supraclavicular fossa

Table 4. Metastasis (M) Staging

M0	No distant metastasis
M1	Distant metastasis

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Author

Arnold C Paulino, MD Professor of Radiation Oncology, Methodist Hospital and Weill-Cornell Medical College; Associate Professor of Pediatrics, Baylor College of Medicine

Arnold C Paulino, MD is a member of the following medical societies: Radiological Society of North America, Children's Oncology Group, American Society of Clinical Oncology, International Society of Paediatric Oncology, American Medical Association, American Radium Society, American Society for Radiation Oncology

Disclosure: Received royalty from Elsevier, Inc for author of book.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Steven K Bergstrom, MD Department of Pediatrics, Division of Hematology-Oncology, Kaiser Permanente Medical Center of Oakland

Steven K Bergstrom, MD is a member of the following medical societies: Alpha Omega Alpha, Children's Oncology Group, American Society of Clinical Oncology, International Society for Experimental Hematology, American Society of Hematology, American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Chief Editor

Cameron K Tebbi, MD Director, Children's Cancer Research Group Laboratories

Cameron K Tebbi, MD is a member of the following medical societies: International Society of Pediatric Oncology

Disclosure: Nothing to disclose.

Additional Contributors

Chrystal U Louis, MD, MPH Assistant Professor, Texas Children's Cancer Center and Hematology Service, Center for Cell and Gene Therapy, Baylor College of Medicine

Disclosure: Received royalty from Cell Medica for other.

Acknowledgements

Samuel Gross, MD Professor Emeritus, Department of Pediatrics, University of Florida College of Medicine; Clinical Professor, Department of Pediatrics, University of North Carolina at Chapel Hill School of Medicine; Adjunct Professor, Department of Pediatrics, Duke University School of Medicine

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.