Prognostic Factors and Long-Term Outcomes of Childhood Nasopharyngeal Carcinoma

BACKGROUND: The authors studied the survival and long-term morbidities of children with nasopharyngeal carcinoma (NPC). METHODS: This was a retrospective review of children with NPC who were treated at St. Jude Children's Research Hospital between 1961 and 2004. Prognostic factors and long term effects of therapy were analyzed. RESULTS: Fifty-nine patients (median age, 14.1 years) were identified. Most were male (66.1%) and black (54.2%) and had lymphoepitheloma (93.2%). Thirty-five patients had stage IV disease (59.3%), 20 patients had stage III disease (33.9%), and 4 patients had stage II disease (6.8%). All patients received radiotherapy (RT) to the primary tumor, and most received cervical RT (98.3%) and chemotherapy (88.1%). The 15-year survival and event-free survival (EFS) rates were 67.2% +/- 7.5% and 63.5% +/- 7.8%, respectively. Five patients (8.5%) developed subsequent malignancies 8.6 to 27 years after NPC diagnosis. EFS was improved in patients who were diagnosed after 1980 (74.8% +/- 10% vs 45.5% +/- 10.1%; P = .031), in patients who had stage III disease compared with patients who had stage IV disease (79.3% +/- 9.6% vs 56.2% +/- 11.8%; P = .049), in patients who received cisplatin (81% +/- 10.7% vs 45.8% +/- 9.7%; P =.013), and in patients who received >= 50 grays of RT (71.4% +/- 9.3% vs 43.8% +/- 11.6%; P=.048). White patients had higher distant failure rates than back patients (41.7% +/- 10.4% vs 15.6 +/- 6.5%; P = .045). The 15-year cumulative incidence (Cl) of any morbidity was 83.7% +/- 5.4%, the CI of sensorineural hearing loss was 52.9% +/- 6.7%, the Cl of primary hypothyroidism was 42.7% +/- 6.6%, and the Cl of growth hormone deficiency (GHD) was 14.1% +/- 4.7%. Dose-response relations were observed between the RT dose and primary hypothyroidism and GHD. CONCLUSIONS: The outcome of children with NPC improved over the past 4 decades with the use of cisplatin-based chemotherapy and higher RT doses. However, many survivors had long-term treatment-related morbidities. Cancer 2011;117:197-206. (C) 2070 American Cancer Society