Rare Cancers in Children – The EXPeRT Initiative: A Report from the European Cooperative Study Group on Pediatric Rare Tumors

Abstract

The low incidence and the heterogeneity of very rare tumors (VRTs) demand for international cooperation. In 2008, EXPeRT (European Cooperative Study Group for Pediatric Rare Tumors) was founded by national groups from Italy, France, United Kingdom, Poland and Germany. The first aims of EXPeRT were to agree on a uniform definition of VRTs and to develop the currently most relevant scientific questions. Current initiatives include international data exchange, retrospective and prospective studies of specific entities, and the development of harmonized and internationally recognized guidelines. Moreover, EXPeRT established a network for expert consultation to assist in clinical decision in VRTs.

Zusammenfassung

Seltene Tumoren zeichnen sich nicht nur durch eine niedrige Inzidenz sondern auch durch ihre ausgeprägte eine klinische und histologische Heterogenität aus. Um die Forschungsaktivitäten auf diesem Gebiet zu unterstützen, wurde 2008 eine internationale Arbeitsgruppe, die European Cooperative Study Group on Pediatric Rare Tumors (EXPeRT), gegründet. In ihr kooperie­ren die Arbeitsgruppen aus Italien, Frankreich, Großbritannien, Polen und Deutschland. Als erstes Ziel wurden eine einheitliche Definition der Seltenen Tumoren entwickelt und wissenschaftliche Fragestellungen formuliert. Aktuelle Initiativen beinhalten den Datenaustausch und gemeinsame retro- und prospektive Analysen für bestimmte Entitäten. Darüber hinaus wird eine Vereinheitlichung der Behandlungsempfehlungen für Seltene Tumoren angestrebt. Auf dieser Grundlage baut EXPeRT ein klinisches und wissenschaftliches Konsiliarnetz auf, das anfragende Kliniken bei anstehenden klinischen Entscheidungen bei Kindern und Jugendlichen mit seltenen Tumoren unterstützt.

Key words

rare tumors - epidemiology - children - network - orphan disease

Schlüsselwörter

seltene Tumoren - Epidemiologie - Netzwerk - Register

*

^* These authors contributed equally to this manuscript.

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