Multimodal Treatment of Nasopharyngeal Carcinoma in Children, Adolescents and Young Adults-Extended Follow-Up of the NPC-2003-GPOH Study Cohort and Patients of the Interim Cohort

Abstract

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.

Nasopharyngeal Carcinoma in Children, Current Treatment Approach

“…[29][30][31][32] TREATMENT PRINCIPLES Over the last decades, and despite the limitations in performing randomized controlled studies in rare pediatric tumors, more prospective studies are being conducted with improved data on the evaluation and treatment of pediatric NPC. 9,10,13,17,18,33,34 Pediatric NPC has a good overall outcome, with more than 80% to 90% of children achieving long-lasting remissions. 11,13,15,35 Combined chemoradiotherapy is the basis of the treatment of pediatric NPC.…”

“…8 The median age at diagnosis for pediatric NPC is between 12 to 15 years. 7,[9][10][11][12][13][14] NPC arises from the deep areas of the head and neck region, and symptoms often become apparent only when there is extensive local infiltration by tumor. This leads in many instances to a delay in diagnosis.…”

“…10,[15][16][17][18] The unique aspects of NPC in children are reflected by the histologic type, mostly undifferentiated World Health Organization type 3, compared with types 1 and 2 that are prevalent in adult NPC, its association with Epstein-Barr virus (EBV) infection, and by its favorable prognosis compared with adult NPC. 11,13,19,20…”

The French FRACTURE database: A way to improve knowledge on management of children with very rare tumors

“…VRTs in children and adolescents represent a heterogeneous group of cancers. Due to their extremely low incidence, knowledge on these tumors is scant and they are the subject of only very few prospective studies; for example, a nasopharyngeal carcinoma study in Germany 3 . Consequently, therapeutic recommendations are difficult to produce, and physicians have to treat VRTs on an individual basis with weak evidence‐based medicine support 4 .…”

“…Due to their extremely low incidence, knowledge on these tumors is scant and they are the subject of only very few prospective studies; for example, a nasopharyngeal carcinoma study in Germany. 3 Consequently, therapeutic recommendations are difficult to produce, and physicians have to treat VRTs on an individual basis with weak evidence-based medicine support. 4 In order to face these difficulties, over the foregoing years, the European Pediatric Oncology Community has developed projects specifically dedicated to VRTs.…”