Nasopharyngeal Cancer Clinical Presentation

  • Author: Arnold C Paulino, MD; Chief Editor: Robert J Arceci, MD, PhD   more...
 
Updated: Apr 26, 2012
 

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).

One study indicated the following symptoms:[4]

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

  • 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.
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Causes

Viral DNA in nasopharyngeal carcinoma has revealed that Epstein-Barr virus (EBV) can infect epithelial cells and is associated with their transformation to cancer.[1] Genetic and environmental factors have been implicated in the development of this disease. A genetic etiology has been considered due to the higher rates of disease within specific ethnic groups, patients with first-degree relatives with the disease, patients with A2 HLA haplotypes, and cytogenetic abnormalities identified within tumor samples.[5, 6] Environmental causes must be considered due to the geographical distribution of the disease, bimodal age distribution, and association seen in patients who consume a large amount of preserved foods and/or salted fish.[7]

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Contributor Information and Disclosures
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: American Medical Association, American Radium Society, American Society for Therapeutic Radiology and Oncology, American Society of Clinical Oncology, Children's Oncology Group, International Society of Paediatric Oncology, and Radiological Society of North America

Disclosure: Nothing to disclose.

Coauthor(s)

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: Nothing to disclose.

Specialty Editor Board

Samuel Gross, MD  Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University

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.

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, American Society of Clinical Oncology, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, and International Society for Experimental Hematology

Disclosure: Nothing to disclose.

Helen SI Chan, MBBS, FRCP(C), FAAP  Associate Senior Scientist, Research Institute; Professor, Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto Faculty of Medicine, Canada

Helen SI Chan, MBBS, FRCP(C), FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Society of Hematology, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD  King Fahd Professor of Pediatric Oncology, Professor of Pediatrics, Oncology and the Cellular and Molecular Medicine Graduate Program, Kimmel Comprehensive Cancer Center at Johns Hopkins University School of Medicine

Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

References

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  2. Greene MH, Fraumeni JF, Hoover R. Nasopharyngeal cancer among young people in the United States: racial variations by cell type. J Natl Cancer Inst. May 1977;58(5):1267-70. [Medline].

  3. Berry MP, Smith CR, Brown TC, Jenkin RD, Rider WD. Nasopharyngeal carcinoma in the young. Int J Radiat Oncol Biol Phys. Apr 1980;6(4):415-21. [Medline].

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  6. 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. Jul 10 2005;115(5):742-6. [Medline].

  7. Yuan JM, Wang XL, Xiang YB, Gao YT, Ross RK, Yu MC. Preserved foods in relation to risk of nasopharyngeal carcinoma in Shanghai, China. Int J Cancer. Feb 1 2000;85(3):358-63. [Medline].

  8. Henle G, Henle W. Epstein-Barr virus-specific IgA serum antibodies as an outstanding feature of nasopharyngeal carcinoma. Int J Cancer. Jan 15 1976;17(1):1-7. [Medline].

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  15. 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. Apr 1998;16(4):1310-1317. [Medline].

  16. International Nasopharynx Cancer Study Group. Preliminary results of a randomized trial comparing neoadjuvant chemotherapy (cisplatin, epirubicin, bleomycin) plus radiotherapy vs. radiotherapy alone in stage IV(> or = N2, M0) undifferentiated nasopharyngeal carcinoma: a positive effect on progres. Int J Radiat Oncol Biol Phys. Jun 1 1996;35(3):463-9. [Medline].

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  20. Wolden SL, Steinherz PG, Kraus DH, Zelefsky MJ, Pfister DG, Wollner N. Improved long-term survival with combined modality therapy for pediatric nasopharynx cancer. Int J Radiat Oncol Biol Phys. Mar 1 2000;46(4):859-64. [Medline].

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  22. Casanova M, Bisogno G, Gandola L, Cecchetto G, DiCataldo A, Basso E, et al. A prospective protocol for nasopharyngeal carcinoma in children and adolescents. The Italian rare tumors in pediatric age (TREP) project. Cancer. 2011;Epub ahead of print:[Medline].

  23. Orbach D, Brisse H, Helfre S, Klijanienko J, Bours D, Mosseri V, et al. Radiation and chemotherapy combination for nasopharyngeal carcinoma in children: Radiotherapy dose adaptation after chemotherapy response to minimize late effects. Pediatr Blood Cancer. Apr 2008;50(4):849-53. [Medline].

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  25. Lee N, Xia P, Quivey JM, Sultanem K, et al. Intensity-modulated radiotherapy in the treatment of nasopharyngeal carcinoma: an update of the UCSF experience. Int J Radiat Oncol Biol Phys. May 1 2002;53(1):12-22. [Medline].

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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
Table 1. AJCC Staging for Nasopharyngeal Cancer
StageTNM
0TisNoM0
IT1N0M0
IIT1N1M0
T2N0M0
T2N1M0
IIIT1N2M0
T2N2M0
T3N0M0
T3N1M0
T3N2M0
IVAT4N0M0
T4N1M0
T4N2M0
IVBAny TN3M0
IVCAny TAny NMI
Table 2. Tumor (T) Staging
TXPrimary tumor cannot be assessed
T0No evidence of primary tumor
TisCarcinoma in situ
T1Tumor confined to the nasopharynx or extends to oropharynx and/or nasal cavity without parapharyngeal extension
T2Tumor with parapharyngeal extension
T3Tumor involves bony structures of skull base and/or paranasal sinuses
T4Tumor 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
NXRegional lymph nodes cannot be assessed
N0No regional lymph node metastasis
N1Unilateral 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
N2Bilateral metastasis in a cervical lymph node (s), less than or equal to 6 cm in greatest dimension, above the supraclavicular fossa
N3Metastasis in a lymph node(s) greater than 6 cm and/or to supraclavicular fossa
N3aGreater than 6 cm in dimension
N3bExtension to supraclavicular fossa
Table 4. Metastasis (M) Staging
M0No distant metastasis
M1Distant metastasis
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