title: A prospective protocol for nasopharyngeal carcinoma in children and adolescents: the Italian Rare Tumors in Pediatric Age (TREP) project.
abstract: BACKGROUND
  Nasopharyngeal carcinoma (NPC) is very rare in childhood. It differs from its adult counterpart in the prevalence of the nonkeratinizing, undifferentiated subtype and by an advanced clinical stage at onset and better chances of survival. The risk of long-term treatment-related toxicity also may be a more important issue in younger individuals.
  
  
  METHODS
  A prospective chemoradiotherapy protocol for pediatric NPC was started in Italy in 2000 within the framework of the Rare Tumors in Pediatric Age (TREP) project. Three courses of cisplatin/5-fluorouracil induction chemotherapy were followed by radiotherapy (doses up to 65 grays) with concomitant cisplatin.
  
  
  RESULTS
  Forty-six patients (ages 9-17 years) were considered eligible for the study over a 10-year period. The ratio of observed to expected cases based on epidemiological data was approximately 1 for both children and adolescents. All but 1 patient had lymph node involvement, and 5 patients had distant metastases. The rate of response to primary chemotherapy was 90%. The 5-year overall and progression-free survival rates were 80.9% and 79.3%, respectively (median follow-up, 62 months). The only statistically significant prognostic variable was the presence or absence of distant metastases. A 65% incidence of late sequelae was reported.
  
  
  CONCLUSIONS
  This study demonstrates the feasibility and efficacy of a prospective protocol even for such rare tumors as pediatric NPC. The use of lower radiotherapy doses than those used in adults did not affect locoregional failure rates. Long-term follow-up will be needed to obtain more information on both survival and treatment sequelae. The next objective will be to establish broader, international prospective cooperation schemes.
authors.affiliation: Foundation for the Research and Cure of Cancer, National Cancer Institute, Milan, Italy.
authors.forename: Michela
authors.initials: M
authors.lastname: Casanova
authors.forename: Gianni
authors.initials: G
authors.lastname: Bisogno
authors.forename: Lorenza
authors.initials: L
authors.lastname: Gandola
authors.forename: Giovanni
authors.initials: G
authors.lastname: Cecchetto
authors.forename: Andrea
authors.initials: A
authors.lastname: Di Cataldo
authors.forename: Eleonora
authors.initials: E
authors.lastname: Basso
authors.forename: Paolo
authors.initials: P
authors.lastname: Indolfi
authors.forename: Paolo
authors.initials: P
authors.lastname: D'Angelo
authors.forename: Francesca
authors.initials: F
authors.lastname: Favini
authors.forename: Paola
authors.initials: P
authors.lastname: Collini
authors.forename: Paolo
authors.initials: P
authors.lastname: Potepan
authors.forename: Andrea
authors.initials: A
authors.lastname: Ferrari
mesh_terms: D000293:Adolescent; D002277:Carcinoma; D059248:Chemoradiotherapy; D002648:Child; D004279:DNA, Viral / Q000032:analysis; D018572:Disease-Free Survival; D005260:Female; D004854:Herpesvirus 4, Human / Q000302:isolation & purification; D006801:Humans; D008297:Male; D000077274:Nasopharyngeal Carcinoma; D009303:Nasopharyngeal Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy* / Q000821:virology; D009367:Neoplasm Staging; D011446:Prospective Studies; D016896:Treatment Outcome
chemical_list: D004279:DNA, Viral