Nasopharyngeal carcinoma in young patients: A systematic review of racial demographics

Objectives: To evaluate patients 25 years of age or younger who were treated for nasopharyngeal carcinoma. Study Design: Retrospective review, systematic literature review. Methods: Nasopharyngeal carcinoma (NPC) is rare in the Western world. In people younger than 30 years, it is even less common, with an incidence of 1 to 2 per million. This retrospective study was designed to analyze the outcomes of patients 25 years of age or younger who were treated for NPC at a tertiary care academic medical center. Between November 1982 and December 2004, 15 patients meeting the above criteria were treated. These patients form the basis for this report. A systematic review of all NPC trials published in the United States since 1990 was done and the results compared with those of our cohort. Results: Young African Americans represented 67% of the 15 cases seen at our institution and between 55% and 67% of the patients in the United States. With a mean follow-up of 5.36 years, disease-free survival and overall survival rates were 67% and 87%, respectively. Locoregional control was excellent after treatment with cisplatin-based combination chemoradiotherapy; however, four patients (27%) developed distant metastasis. Despite this, no patients died of disease. Toxicity was significant but manageable, and retreatment met with good success. Conclusions: In patients 25 years of age or younger, NPC is more common in African Americans. This may represent a genetic and racial predisposition of this unusual disease in the United States. Late-stage presentations and distant recurrences are common. Platinum-based combination chemoradiotherapy and aggressive management of metastatic disease, however, is associated with good long-term survival. Further study of the possibly changing epidemiology and racial genetics of this unusual tumor is warranted.