Nasopharyngeal carcinoma in young patients: a systematic review of racial demographics.
OBJECTIVE
To evaluate patients 25 years of age or younger who were treated for nasopharyngeal carcinoma.


METHODS
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.
Cannon
Trinitia
T
Department of Otolaryngology/Head and Neck Surgery, University of North Carolina at Chapel Hill School of Medicine, Chapel Hill, North Carolina 27599-0001, USA. tcannon@unch.unc.edu
Zanation
Adam M
AM
Lai
Victor
V
Weissler
Mark C
MC
D000293:Adolescent; D000328:Adult; D001741:Black or African American; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D009303:Nasopharyngeal Neoplasms / Q000453:epidemiology* / Q000401:mortality / Q000628:therapy; D044469:Racial Groups; D012189:Retrospective Studies; D015996:Survival Rate; D014481:United States / Q000453:epidemiology