Nasopharyngeal carcinoma in children: retrospective review of 50 patients.

PURPOSE: To report a retrospective analysis of epidemiologic, clinical, and therapeutic aspects of 50 children with newly diagnosed nasopharyngeal carcinoma who were treated in a single institution over a period of 18 years. METHODS AND MATERIALS: Thirty-two male and 18 female children ranging from 5 to 16 years, accounted for 7.2% of all nasopharyngeal carcinoma cases and 52% of childhood nasopharyngeal malignancies. Histopathology was World Health Organization Type 3 carcinoma in 45, World Health Organization Type 2 in 4, and World Health Organization Type 1 in one patient. Two of the patients had missing information for staging and treatment evaluation. Disease extent was T1 (n = 4), T2 (n = 9), T3 (n = 21), and T4 (n = 14); N0 (n = 1), N1 (n = 6), N2 (n = 12), and N3 (n = 29). Six patients had base of skull invasion, two had cranial nerve palsies, and six had both. One patient had M1 disease on admission. Twenty-three patients were treated with irradiation only. Thirteen patients received adjuvant, and 12 had neoadjuvant chemotherapy in addition to radiotherapy. Patients received 50-72 Gy to the primary tumor and involved nodes, and 45-50 Gy to uninvolved regions. Chemotherapy consisted of combinations including cisplatin, bleomycin, epirubicin, 5-fluoroucil, and cyclophosphamide.
RESULTS: Thirty-eight (79%) patients attained locoregional control. Overall, 22 patients are alive without relapse 6-195 months from diagnosis. Thirteen patients had 21 relapses, at local and/or regional sites (43%), distant sites (48%), or both (9%). The median time for first relapse was 8 months. Overall, the 5-and 10-year survival rates were 52 and 52%, respectively, and the failure-free survival rates were both 53%. The results of three distinct treatments given in subsequent time periods were not statistically different. Three second malignancies occurred 33-156 months following nasopharyngeal carcinoma diagnosis.
CONCLUSION: In the current series, nasopharyngeal carcinoma patients under the age of 16 accounted for 7.2% of all nasopharyngeal carcinoma cases. Whereas the impact of chemotherapy on long-term survival remains to be determined by randomized studies, the results suggest that more effective treatment regimens and long-term follow-up are necessary for children with nasopharyngeal carcinoma.