title: Treatment results of 165 pediatric patients with non-metastatic nasopharyngeal carcinoma: a Rare Cancer Network study.
abstract: OBJECTIVE
  This Rare Cancer Network (RCN) study was performed in pediatric nasopharyngeal carcinoma (PNPC) patients to evaluate the optimal dose of radiotherapy and to determine prognostic factors.
  
  
  METHODS
  The study included 165 patients with the diagnosis of PNPC treated between 1978 and 2003. The median age was 14 years. There were 3 (1.8%) patients with stage I, 1 (0.6%) with IIA, 10 (6.1%) with IIB, 60 (36.4%) with III, 44 (26.7%) with IVA, and 47 (29%) with IVB disease. While 21 (12.7%) patients were treated with radiotherapy (RT) alone, 144 (87.3%) received chemotherapy and RT. The median follow-up time was 48 months.
  
  
  RESULTS
  The actuarial 5-year overall survival (OS) was 77.4% (95% CI: 70.06-84.72), whereas the actuarial 5-year disease-free survival (DFS) rate was 68.8% (95% CI: 61.33-76.31). In multivariate analysis, unfavorable factors were age >14 years for LRC (p=0.04); male gender for DMFS (p=0.03); T3/T4 disease for LRFS (p=0.01); and N3 disease for DFS (p=0.002) and OS (p=0.002); EBRT dose of less than 66 Gy for LRFS (p=0.02) and LRRFS (p=0.0028); and patients treated with RT alone for LRFS (p=0.0001), LRRFS (p=0.007) and DFS (p=0.02).
  
  
  CONCLUSIONS
  Our results support the current practice of using combined radiation and chemotherapy for optimal treatment of NPC. However, research should be encouraged in an attempt to reduce the potential for long-term sequelae in pediatric patients given their relatively favorable prognosis and potential for longevity.
authors.affiliation: Hacettepe University, Oncology Institute, Ankara, Turkey. eozyar@hacettepe.edu.tr
authors.forename: Enis
authors.initials: E
authors.lastname: Ozyar
authors.forename: Ugur
authors.initials: U
authors.lastname: Selek
authors.forename: Siddihartha
authors.initials: S
authors.lastname: Laskar
authors.forename: Omer
authors.initials: O
authors.lastname: Uzel
authors.forename: Yavuz
authors.initials: Y
authors.lastname: Anacak
authors.forename: Miriam
authors.initials: M
authors.lastname: Ben-Arush
authors.forename: Sopiha
authors.initials: S
authors.lastname: Polychronopoulou
authors.forename: Fadime
authors.initials: F
authors.lastname: Akman
authors.forename: Suzanne L
authors.initials: SL
authors.lastname: Wolden
authors.forename: Süreyya
authors.initials: S
authors.lastname: Sarihan
authors.forename: Robert C
authors.initials: RC
authors.lastname: Miller
authors.forename: Mahmut
authors.initials: M
authors.lastname: Ozsahin
authors.forename: Ufuk
authors.initials: U
authors.lastname: Abacioğlu
authors.forename: Margarita
authors.initials: M
authors.lastname: Martin
authors.forename: Murat
authors.initials: M
authors.lastname: Caloglu
authors.forename: Luciano
authors.initials: L
authors.lastname: Scandolaro
authors.forename: Eva
authors.initials: E
authors.lastname: Szutowicz
authors.forename: Ibtisam Lale
authors.initials: IL
authors.lastname: Atahan
mesh_terms: D000293:Adolescent; D000367:Age Factors; D002648:Child; D003131:Combined Modality Therapy / Q000009:adverse effects / Q000379:methods; D018572:Disease-Free Survival; D019583:Dose Fractionation, Radiation; D004812:Epidemiologic Methods; D005260:Female; D006801:Humans; D008297:Male; D009303:Nasopharyngeal Neoplasms / Q000188:drug therapy / Q000401:mortality / Q000532:radiotherapy*; D009364:Neoplasm Recurrence, Local; D011379:Prognosis; D035583:Rare Diseases / Q000188:drug therapy / Q000401:mortality / Q000532:radiotherapy*; D012737:Sex Factors; D016896:Treatment Outcome