Nasopharyngeal Cancer 

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

Background

Nasopharyngeal carcinoma is a rare tumor arising from the epithelium of the nasopharynx. It accounts for approximately 1% of all childhood malignancies. Whereas almost all adult nasopharyngeal cancers are carcinomas, only 35-50% of nasopharyngeal malignancies are carcinomas in children. In the pediatric population, additional nasopharyngeal malignancies include rhabdomyosarcomas or lymphomas.

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Pathophysiology

The detection of the Epstein-Barr virus (EBV) nuclear antigen and viral DNA in nasopharyngeal carcinoma has revealed that EBV can infect epithelial cells and is associated with their malignant transformation.[1] Copies of the EBV genome have been found in cells of preinvasive lesions, suggesting that it is directly related to the process of transformation.

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Epidemiology

Frequency

United States

Although the incidence varies according to geographic location, approximately 1 in every 100,000 children are diagnosed annually in North America.

International

The disease is far more common in children of Southeast Asian and Northern African descent, with an incidence of 8-25 in every 100,000 children annually.

Mortality/Morbidity

When radiotherapy is used alone, survival rates range from 40-50%. Use of combination radiation therapy and chemotherapy allows long-term survival rates of 55-80%.

Race

In the United States, the incidence of nasopharyngeal carcinoma is increased among black teenagers.[2] Children of Asian, Middle Eastern, and Northern African descent are also more commonly affected.

Sex

A male preponderance is observed. The male-to-female ratio is approximately 2:1.

Age

Nasopharyngeal carcinoma has a bimodal age distribution. A small peak is observed in late childhood, and a second peak occurs in people aged 50-60 years. Childhood nasopharyngeal carcinoma is usually a disease of adolescence.[3]

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Proceed to Clinical Presentation
 
 
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

  1. 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. Sep 14 1995;333(11):693-8. [Medline].

  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].

  4. Sham JS, Poon YF, Wei WI, Choy D. Nasopharyngeal carcinoma in young patients. Cancer. Jun 1 1990;65(11):2606-10. [Medline].

  5. 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. Jan-Feb 1999;19(1B):661-5. [Medline].

  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].

  9. Gastpar H, Wilmes E, Wolf H. Epidemiologic, etiologic and immunologic aspects of nasopharyngeal carcinoma [NPC]. J Med. 1981;DA - 19820212(4):257-84. [Medline].

  10. Leung SF, Zee B, Ma BB, Hui EP, Mo F, Lai M, et al. Plasma Epstein-Barr viral deoxyribonucleic acid quantitation complements tumor-node-metastasis staging prognostication in nasopharyngeal carcinoma. J Clin Oncol. Dec 1 2006;24(34):5414-8. [Medline].

  11. Ingersoll L, Woo SY, Donaldson S, et al. Nasopharyngeal carcinoma in the young: a combined M.D. Anderson and Stanford experience. Int J Radiat Oncol Biol Phys. Oct 1990;19(4):881-7. [Medline].

  12. Ayan I, Altun M. Nasopharyngeal carcinoma in children: retrospective review of 50 patients. Int J Radiat Oncol Biol Phys. Jun 1 1996;35(3):485-92. [Medline].

  13. Cooper JS, Cohen R, Stevens RE. A comparison of staging systems for nasopharyngeal carcinoma. Cancer. Jul 15 1998;83(2):213-9. [Medline].

  14. Pharynx. In: Edge SB, Byrd dR, Compton cc, Fritz AG, Greene FL, Trotti A. AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer; 2010:41-56.

  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].

  17. Chan AT, Teo PM, Leung TW, Johnson PJ. The role of chemotherapy in the management of nasopharyngeal carcinoma. Cancer. Mar 15 1998;82(6):1003-12. [Medline].

  18. Douglass EC, Fontanesi J, Ribiero RC. Improved long-term disease free survival in nasopharyngeal carcinoma in childhood and adolescence. Proc ASCO. 1996;15:467.

  19. Rodriguez-Galindo C, Wofford M, Castleberry RP, et al. Preradiation chemotherapy with methotrexate, cisplatin, 5-fluorouracil, and leucovorin for pediatric nasopharyngeal carcinoma. Cancer. Feb 15 2005;103(4):850-7. [Medline].

  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].

  21. Ozyar E, Selek U, Laskar S, et al. Treatment results of 165 pediatric patients with non-metastatic nasopharyngeal carcinoma: a Rare Cancer Network study. Radiother Oncol. Oct 2006;81(1):39-46. [Medline].

  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].

  24. Louis CU, Paulino AC, Gottschalk S, et al. A single institution experience with pediatric nasopharyngeal carcinoma: high incidence of toxicity associated with platinum-based chemotherapy plus IMRT. J Pediatr Hematol Oncol. Jul 2007;29(7):500-5. [Medline].

  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].

  26. Laskar S, Bahl G, Muckaden M, Pai SK, Gupta T, Banavali S, et al. Nasopharyngeal carcinoma in children: comparison of conventional and intensity-modulated radiotherapy. Int J Radiat Oncol Biol Phys. Nov 1 2008;72(3):728-36. [Medline].

  27. Delanian S, Balla-Mekias S, Lefaix JL. Striking regression of chronic radiotherapy damage in a clinical trial of combined pentoxifylline and tocopherol. J Clin Oncol. Oct 1999;17(10):3283-90. [Medline].

  28. 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. Sep 1 1996;36(2):281-9. [Medline].

  29. Teo PM, Chan AT, Lee WY, Leung TW, Johnson PJ. Enhancement of local control in locally advanced node-positive nasopharyngeal carcinoma by adjunctive chemotherapy. Int J Radiat Oncol Biol Phys. Jan 15 1999;43(2):261-71. [Medline].

  30. 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. Sep 1 1997;80(5):951-9. [Medline].

  31. Serin M, Erkal HS, Elhan AH, Cakmak A. Nasopharyngeal carcinoma in childhood and adolescence. Med Pediatr Oncol. Dec 1998;31(6):498-505. [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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