Very rare tumours in paediatric age - From 'Tumori Rari in Età Pediatrica' to the European cooperative study group for paediatric rare tumours

Abstract

Very rare tumours (VRTs) in paediatric age are a heterogeneous group of cancers very rarely encountered in daily practice, even in large paediatric oncology centres. Some of them are typical of paediatric age, such as pleuropulmonary blastoma or pancreatoblastoma; others are typically found in adulthood, such as carcinomas and melanoma. With the objective of improving the research on, and management of, paediatric VRTs, a national study group was founded and the Tumori Rari in Età Pediatrica (Rare Tumours in Paediatric Age [TREP]) project was launched in Italy in 2000. For the purposes of this project, VRTs have been defined as "any solid malignancy characterized by an annual incidence of <2 cases/million children and not considered in other clinical trials". From January 2000 to December 2011, 652 patients <18 years of age were registered in the TREP database. This article presents the experience gathered so far and underlines the need to develop international collaborations dedicated to paediatric VRTs. With this aim, national groups from Italy, Germany, France, Poland and the UK have created, in June 2008, a new collaborative group named European Cooperative Study Group for Paediatric Rare Tumours (EXPeRT). © TOUCH BRIEFINGS 2012.