Defining and listing very rare cancers of paediatric age: consensus of the Joint Action on Rare Cancers in cooperation with the European Cooperative Study Group for Pediatric Rare Tumors

Research output: Contribution to journalArticle


  • Andrea Ferrari
  • Ines B. Brecht
  • Gemma Gatta
  • Dominik T. Schneider
  • Daniel Orbach
  • Giovanni Cecchetto
  • Jan Godzinski
  • Yves Reguerre
  • Ewa Bien
  • Teresa Stachowicz-Stencel
  • Michael Ost
  • Chiara Magni
  • Gilles Vassal
  • Maura Massimino
  • Andrea Biondi
  • Gianni Bisogno
  • Annalisa Trama

External organisations

  • Fondazione IRCCS Istituto Nazionale dei Tumori, Milan
  • Universitat Tubingen
  • Dortmund Municipal Hospital
  • Institut Curie
  • Padova University Hospital
  • Marciniak Hospital
  • Centre Hospitalier Universitaire
  • Medical University
  • European Society for Paediatric Oncology (SIOPE Europe)
  • Universite Paris Sud
  • University of Milano-Bicocca


Although all tumours are rare in childhood, there are some particularly rare paediatric cancers which have not benefited from advances made by the international paediatric oncology network. To establish a shared definition and produce a list of these entities, the European Union Joint Action on Rare Cancers (JARC) promoted a consensus effort. The definition was based on the incidence rates estimated using the information network on rare cancers (RARECAREnet) database, pooling data from 94 population-based cancer registries and 27 countries. The RARECAREnet list of cancers was used to estimate the incidence rates. This list groups cancers by combining the International Classification of Diseases for Oncology, third edition, morphology and topography codes. According to the consensus, very rare paediatric cancers were identified as those with an annual incidence <2/1000000 and corresponded to 11% of all cancers in patients aged 0–14 years. Two subgroups were identified: tumour types typical of childhood (i.e. hepatoblastoma, pleuropulmonary blastoma, pancreatoblastoma) and those typical of adult age (i.e. carcinomas, melanoma). The threshold of 2/1000000 could also be adopted in populations aged 0–19 years: in this case, three tumour types had an incidence rate which was >2/1000000 (i.e. thyroid and testicular cancers and skin melanoma), but the consensus experts considered them as ‘very rare’ according to their clinical needs (e.g. shortage of knowledge and clinical expertise as the other rare paediatric cancers). The JARC consensus produced a definition and a list of very rare paediatric cancers which may represent a starting point for prioritising research on these tumours, based on data and patients’ clinical needs.


Original languageEnglish
Pages (from-to)120-126
Number of pages7
JournalEuropean Journal of Cancer
Early online date19 Feb 2019
Publication statusPublished - Mar 2019


  • Very rare paediatric cancers, Consensus, Cancer registries, Incidence

ASJC Scopus subject areas