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Nasopharyngeal Cancer

  • Author: Arnold C Paulino, MD; Chief Editor: Robert J Arceci, MD, PhD  more...
 
Updated: Jan 29, 2015
 

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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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: 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.

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: Received royalty from Cell Medica for other.

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

Robert J Arceci, MD, PhD Director, Children’s Center for Cancer and Blood Disorders, Department of Hematology/Oncology, Co-Director of the Ron Matricaria Institute of Molecular Medicine, Phoenix Children’s Hospital; Editor-in-Chief, Pediatric Blood and Cancer; Professor, Department of Child Health, University of Arizona College of Medicine

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

Disclosure: Nothing to disclose.

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.

References
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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
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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