ELSEVIER Int. J. Radiation Oncology Biol. Phys., Vol. 35, No. 3, pp. 485-492, 1996 Copyright © t996 Elsevier Science Inc. Printed in the USA. All rights reserved 0360-3016/96 $15.00 + .00 PII: S0360-3016(96) 00018-1 • Clinical Original Contribution NASOPHARYNGEAL CARCINOMA IN CHILDREN: RETROSPECTIVE REVIEW OF 50 PATIENTS iNCi AYAN, M.D. * AND MUSA ALTUN, M.D. * • Department of Pediatric Oncology and t Department.of Radiation Oncology, Institute of Oncology, University of Istanbul, Istanbul, Turkey 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); NO (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-flouroucil, and cyclophosphamide. Results: Thirty-eight (79%) patients attained locoregionel 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. Childhood nasopharyngeal carcinoma, Radiotherapy, Chemotherapy, Second malignancy. INTRODUCTION The incidence of childhood nasopharyngeal carcinoma (NPC) varies greatly according to racial and geographical factors ( 13, 34). In general it represents less than 1% of all childhood malignant tumors but constitutes 20 to 50% of pediatric malignancies of the nasopharynx (13, 19, 31 ). Although the mean age at diagnosis is between 40- 50 years, NPC may occur at almost any age (2, 14). A bimodal age incidence graph with a first small peak in late childhood is observed in some populations (7, 10, 13, 23, 38). Children with NPC almost always have the undifferen- tiated variant (World Health Organization [WHO] Type 3) of disease, which is closely related to a higher rate of advanced locoregional disease and distant metastasis ( 15, 19, 31 ). Despite a high incidence of advanced locoregio- nal disease the overall survival rates do not significantly differ from adults, and the 5-year disease-free survival rate is between 29-60% in reported pediatric series (7, 19, 25, 31). Standard therapy for NPC in children has generally followed the quidelines established for adults, which con- sist of high dose radiation to nasopharynx and involved cervical nodal regions as well as moderate dose to unin- volved cervical nodal sites. Unfortunately, high dose ra- Reprint requests to: |nci Ayan, M.D., Institute of Oncology, University of lstanbul, ~apa 34390, |stanbul, Turkey. Acknowledgements--We wish to thank Ayten Cangir, M.D., for her helpful discussions. We also thank Nijad Bilge, M.D., Osman Aldemir, M.D., Mtinir Kinay, M.D., Nihat Ayan, M.D., Emin Darendeliler, M.D., Rejin Kebudi, M.D., Omer GOrgtin, M.D., Erkan Topuz, M.D., Haluk Onat, M.D., and Rian Die,i, Ph.D,, for their contributions, and Fadime O~uz for preparing the manuscript. Accepted for publication 29 December 1995. 485