title: Nasopharyngeal carcinoma in children and adolescents in an endemic area: a report of 185 cases.
abstract: BACKGROUND
  This study aimed to demonstrate the clinical and therapeutic features of nasopharyngeal carcinoma (NPC) in children and adolescents in Southern China, an endemic area.
  
  
  METHODS
  A total of 185 newly diagnosed NPC patients younger than 21 years old in the Sun Yat-sen University Cancer Center from 1993 to 2011 were retrospectively analyzed. Overall survival (OS) rate estimates and Kaplan-Meier survival curves were calculated. Cox proportional hazard ratios (HRs) were used to identify independent prognostic factors for survival. Chi-square test was used to compare the incidence of sequelae and the stage distribution between different subgroups.
  
  
  RESULTS
  Most patients were male (71.4%). The main presenting symptoms were neck mass (44.9%), tinnitus/hearing loss (36.2%), bloody nasal discharge (22.7%), headache (22.2%), and nasal obstruction (20.0%). Stage I, II, III, and IV patients accounted for 1.1%, 8.1%, 43.8%, and 47.0%, respectively, of the total number of patients included in the study. All patients were treated by radiotherapy: 39 Gy-84 Gy to primary tumors (median, 68 Gy) and 36 Gy-74 Gy to cervical lymph nodes (median, 60 Gy); 84.3% of the patients were treated by chemotherapy either. The complete response rate was 94.1%. The 5-, 10-, and 15-year survival rates were 78% ± 4%, 70% ± 5%, and 66% ± 6%, respectively. Tumor node metastasis (TNM) stage was the statistically significant predictor of distal metastasis and OS. Distal metastasis was the major pattern of treatment failure. The main long-term complications of therapy were xerostomia (47.0%), hearing loss (28.1%), neck fibrosis (24.3%), trismus (12.4%), glossolalia (7.0%), and radiation encephalopathy (5.4%). The incidences of these morbidities were significantly higher in the high radiation dose (more than the median) group than in the low radiation dose group (less than or equal to the median), while no differences in survival were observed.
  
  
  CONCLUSIONS
  In spite of the majority of patients diagnosed at the advanced stage, children and adolescents with NPC had excellent survival except metastatic disease. The TNM stage was the most relevant prognostic factor. A higher radiation dose (>68 Gy) could not improve survival but could increase long-term morbidities.
authors.affiliation: VIP Region, Sun Yat-sen University Cancer Center, 651 Dongfeng East Road, Guangzhou 510060, China.
authors.forename: Zheng
authors.initials: Z
authors.lastname: Yan
authors.forename: Liangping
authors.initials: L
authors.lastname: Xia
authors.forename: Yuanyuan
authors.initials: Y
authors.lastname: Huang
authors.forename: Ping
authors.initials: P
authors.lastname: Chen
authors.forename: Li
authors.initials: L
authors.lastname: Jiang
authors.forename: Bei
authors.initials: B
authors.lastname: Zhang
mesh_terms: D000293:Adolescent; D002277:Carcinoma; D002648:Child; D002675:Child, Preschool; D002681:China / Q000453:epidemiology; D018572:Disease-Free Survival; D019353:Endemic Diseases; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008198:Lymph Nodes / Q000473:pathology*; D008297:Male; D000077274:Nasopharyngeal Carcinoma; D009303:Nasopharyngeal Neoplasms / Q000175:diagnosis / Q000453:epidemiology* / Q000532:radiotherapy*; D009361:Neoplasm Invasiveness / Q000473:pathology; D009367:Neoplasm Staging; D016016:Proportional Hazards Models; D011879:Radiotherapy Dosage; D012189:Retrospective Studies; D018570:Risk Assessment; D016019:Survival Analysis; D016896:Treatment Outcome
keywords: Children and adolescents; Morbidity; Nasopharyngeal carcinoma; Radiotherapy; Survival