Abstract

Simple SummaryMultimodal treatment of nasopharyngeal carcinoma (NPC) in children and young adults with induction chemotherapy, followed by radiochemotherapy and interferon-β (IFN-β) maintenance, has been successfully applied in studies NPC-91 and NPC-2003 of the German Society of Pediatric Oncology and Hematology (GPOH). We, here, present updated survival rates of the NPC-2003 study cohort after longer follow-up and include 21 additional patients recruited after closure of the study and treated as per the NPC-2003 study protocol (interim cohort) in our survival analysis. Survival rates remain high after extended follow-up and in the larger cohort with EFS and OS of 94% and 97%, respectively, reinforcing the high antitumor efficacy of this multimodal treatment concept. Seven patients with CR after induction therapy received a reduced radiation dose of 54 Gy, and none of them relapsed. Thus, the reduction of radiation dose seems feasible and has the potential to reduce treatment-related late effects in this vulnerable population.Nasopharyngeal carcinoma (NPC) in children and young adults has been treated within two consecutive prospective trials in Germany, the NPC-91 and the NPC-2003 study of the German Society of Pediatric Oncology and Hematology (GPOH). In these studies, multimodal treatment with induction chemotherapy, followed by radio (chemo)therapy and interferon-beta maintenance, yielded promising survival rates even after adapting total radiation doses to tumor response. The outcome of 45 patients in the NPC-2003 study was reassessed after a median follow-up of 85 months. In addition, we analyzed 21 further patients after closure of the NPC-2003 study, recruited between 2011 and 2017, and treated as per the NPC-2003 study protocol. The EFS and OS of 66 patients with locoregionally advanced NPC were 93.6% and 96.7%, respectively, after a median follow-up of 73 months. Seven patients with CR after induction therapy received a reduced radiation dose of 54 Gy; none relapsed. In young patients with advanced locoregional NPC, excellent long-term survival rates can be achieved by multimodal treatment, including interferon-beta. Radiation doses may be reduced in patients with complete remission after induction chemotherapy and may limit radiogenic late effects.

Highlights

  • Nasopharyngeal carcinoma (NPC) is a rare neoplasm in children and adolescents, with a reported incidence of 0.4–0.6 per 1,000,000 persons between 10 and 17 years of age in Germany [1]

  • As the NPC-2003-German Society of Pediatric Oncology and Hematology (GPOH) study cohort had been characterized in detail before [4], we focus here on the interim patients recruited between 2011 and 2017

  • This has to be interpreted in view of the lower radiation doses applied in the NPC-GPOH studies when compared to other pediatric NPC protocols [3,4,17,18]

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Summary

Introduction

Nasopharyngeal carcinoma (NPC) is a rare neoplasm in children and adolescents, with a reported incidence of 0.4–0.6 per 1,000,000 persons between 10 and 17 years of age in Germany [1]. Successful treatment of NPC in children and adolescents in Germany has been mainly achieved through the implementation of two consecutive prospective trials on behalf of the German Society of Pediatric Oncology and Hematology (GPOH), the NPC-91 and the NPC-2003 study [3,4]. In these studies, multimodal treatment combining induction chemotherapy, followed by concomitant radio (chemo)therapy, and maintenance with interferon-beta (IFN-β), yielded high survival rates. In the NPC-91 study, 59 patients ≤ 25 years of age were included.

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