Ben-Ami Tal, Kontny Udo, Surun Aurore, Brecht Ines B, Almaraz Ricardo López, Dragomir Monica, Pourtsidis Apostolos, Casanova Michela, Fresneau Brice, Bisogno Gianni, Schneider Dominik T, Reguerre Yves, Bien Ewa, Stachowicz-Stencel Teresa, Österlundh Gustaf, Wygoda Marc, Janssens Geert O, Zsiros József, Jehanno Nina, Brisse Herve J, Gandola Lorenza, Christiansen Hans, Claude Line, Ferrari Andrea, Rodriguez-Galindo Carlos, Orbach Daniel
Pediatric Hematology-Oncology Unit, Kaplan Medical Center, Faculty of Medicine, Hebrew University of Jerusalem, Rehovot, Israel.
Division of Pediatric Hematology Oncology and Stem Cell Transplantation, Medical Faculty, RWTH Aachen University, Aachen, Germany.
Pediatr Blood Cancer. 2021 Jun;68 Suppl 4:e29018. doi: 10.1002/pbc.29018. Epub 2021 Apr 12.
Nasopharyngeal carcinoma (NPC) is a rare pediatric tumor. Collaborative studies performed over the last decades showed improved results compared to historical data, but standardized guidelines for diagnosis and management of pediatric NPC are still unavailable. This study presents a European consensus guideline for the diagnosis and treatment of pediatric NPC developed by the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT). Main recommendations include induction chemotherapy with cisplatin and 5-flurouracil, concomitant chemoradiotherapy in advanced disease, and to consider maintenance treatment with interferon beta (IFN-β) for selected high-risk patients. Dose adjustments of radiotherapy based on response to induction chemotherapy may decrease the rates of long-term treatment-related complications that affect most of the survivors.
鼻咽癌(NPC)是一种罕见的儿童肿瘤。过去几十年进行的合作研究显示,与历史数据相比结果有所改善,但仍没有针对儿童鼻咽癌诊断和管理的标准化指南。本研究提出了由欧洲儿童罕见肿瘤合作研究组(EXPeRT)制定的欧洲儿童鼻咽癌诊断和治疗共识指南。主要建议包括使用顺铂和5-氟尿嘧啶进行诱导化疗,对晚期疾病进行同步放化疗,并考虑对选定的高危患者使用干扰素β(IFN-β)进行维持治疗。根据诱导化疗反应调整放疗剂量可能会降低影响大多数幸存者的长期治疗相关并发症的发生率。