title: Prognostic factors and long-term outcomes of childhood nasopharyngeal carcinoma.
abstract: 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 lymphoepithelioma (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 black patients (41.7% ± 10.4% vs 15.6 ± 6.5%; P = .045). The 15-year cumulative incidence (CI) of any morbidity was 83.7% ± 5.4%, the CI of sensorineural hearing loss was 52.9% ± 6.7%, the CI of primary hypothyroidism was 42.7% ± 6.6%, and the CI 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.
authors.affiliation: Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee, USA.
authors.forename: Daniel Ka Leung
authors.initials: DK
authors.lastname: Cheuk
authors.forename: Catherine A
authors.initials: CA
authors.lastname: Billups
authors.forename: Michael G
authors.initials: MG
authors.lastname: Martin
authors.forename: Cynthia R
authors.initials: CR
authors.lastname: Roland
authors.forename: Raul C
authors.initials: RC
authors.lastname: Ribeiro
authors.forename: Matthew J
authors.initials: MJ
authors.lastname: Krasin
authors.forename: Carlos
authors.initials: C
authors.lastname: Rodriguez-Galindo
mesh_terms: D000293:Adolescent; D000328:Adult; D002277:Carcinoma; D002648:Child; D015897:Comorbidity; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D006319:Hearing Loss, Sensorineural / Q000209:etiology; D006801:Humans; D008297:Male; D000077274:Nasopharyngeal Carcinoma; D009303:Nasopharyngeal Neoplasms / Q000628:therapy; D016609:Neoplasms, Second Primary / Q000453:epidemiology; D011379:Prognosis; D011832:Radiation Injuries / Q000453:epidemiology; D011878:Radiotherapy / Q000009:adverse effects; D016896:Treatment Outcome