A prospective protocol for nasopharyngeal carcinoma in children and adolescents

Abstract

on behalf of the Rare Tumors in Pediatric Age Group 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.

Overview of Carcinomas in Adolescents: A Descriptive Study Over Twenty Years

“…Concerning NPC, survival was quite similar to those previously reported (76.5% 5y-OS vs. 71-81%) [14][15][16][17]. The main indicator for poor prognosis was distant metastases which were present in only 12% of cases as compared to 2.7% to 27% in previous series.…”

“…Only 2/7 patients with FL-HCC and 1/5 HCC were alive with a follow-up of 47, 77 and 74 months but after multiple recurrences treated with various combined therapies (multiple surgeries, chemotherapy, hyperthermic intraperitoneal chemotherapy, inhibitor tyrosine kinase, radiofrequency). The median survival of patients was 26 months (12-78) for FL-HCC and 16.5 months (8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25) for HCC.…”

5-Fluorouracil causes severe CNS demyelination by disruption of TCF7L2/HDAC1/HDAC2 complex in adolescent mice

“…In recent years, clinical studies have demonstrated effectiveness of 5-FU on pediatric tumors, such as low-grade gliomas, nasopharyngeal carcinoma and hepatoblastoma (Casanova et al, 2012;Kupeli et al, 2006;Lee et al, 2006;Rodriguez-Galindo et al, 2005). The previous studies, including experimental and clinical reports, have suggested that 5-FU could result in oligodendrocytes (OLs) injury and reversible white-matter degeneration in adult patients (Cho et al, 2004;Fujimoto et al, 2006;Han et al, 2008;Ishibashi et al, 2004;Newton 2012;Sioka and Kyritsis, 2009).…”

Induction Chemotherapy Followed by Radiotherapy versus Concurrent Chemoradiotherapy in elderly patients with nasopharyngeal carcinoma: finding from a propensity-matched analysis

“…Nasopharyngeal carcinoma (NPC) is a special head and neck cancer in terms of its epidemiology, etiology, clinical presentation, and prognostic factors [1]. The incidence of NPC is increasing with age in the endemic areas, with a peak and subsequently an earlier decline in age-incidence (in middle-age, ages 45–60 years) than seen in any low-risk population [2].…”