A prospective protocol for nasopharyngeal carcinoma in children and adolescents: The Italian Rare Tumors in Pediatric Age (TREP) project

Michela Casanova, Gianni Bisogno, Lorenza Gandola, Giovanni Cecchetto, Andrea Di Cataldo, Eleonora Basso, Paolo Indolfi, Paolo D'Angelo, Francesca Favini, Paola Collini, Paolo Potepan, Andrea Ferrari

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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. Cancer 2011. © 2011 American Cancer Society. The current results indicate the feasibility and efficacy of a prospective chemoradiotherapy protocol for patients with pediatric nasopharyngeal carcinoma that was developed between 2000 and 2009 within the framework of the Italian Rare Tumors in Pediatric Age (TREP) project.

Original languageEnglish
Pages (from-to)2718-2725
Number of pages8
JournalCancer
Volume118
Issue number10
DOIs
Publication statusPublished - May 15 2012

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Keywords

  • chemotherapy
  • children and adolescents
  • nasopharyngeal carcinoma
  • radiotherapy
  • rare pediatric tumors
  • Rare Tumors in Pediatric Age (TREP) project
  • sequelae

ASJC Scopus subject areas

  • Cancer Research
  • Oncology

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