Background: Nasopharyngeal carcinoma is a rare tumor arising from the epithelium of the nasopharynx. It accounts for fewer than 1% of cases of childhood malignancy. Whereas almost all adult nasopharyngeal cancers are carcinomas, only 20-35% of nasopharyngeal malignancies are carcinomas in children. In the pediatric population, most nasopharyngeal malignancies are rhabdomyosarcomas or lymphomas.

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 transformation to cancer. Clonal EBV DNA is found in cells in preinvasive lesions, suggesting that it is directly related to the process of transformation.

Frequency:

•           In the US: Incidence is variable according to geographic location. Incidence is approximately 1 in every 100,000 children annually among white children in North America and Europe. 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.

•           Children of Asian and Northern African descent are also more commonly affected.

Sex: A male preponderance exists. 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.

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