Objective 3:
Tumour biology
To increase knowledge of tumour biology and
speed up translation from basic research to clinical
care to benefit patients.
Cancers in adults result from processes that have multiple
steps, mainly following exposure to external carcinogens
(tobacco, alcohol, UV, diet, etc.) and often progression
over many years.
In contrast, paediatric cancers develop early in life and over a much shorter time period,
suggesting that fewer and stronger events are required for progression. They are rare, andmost
show fewer genetic defects and a lower genetic complexity as compared to adult cancers [13].
Major progress has been made in understanding paediatric tumour biology, leading to the
discovery of unique cancer hallmarks that are also involved in cancer formation in adults,
such as the RB1 gene in retinoblastoma and, more recently, Histones H3 mutations in diffuse
intrinsic pontine gliomas [12]. These advances have already resulted in new classification of
several diseases. Additionally, the role of the immune system in controlling tumour growth is
now well-established in many adult cancers, and the challenge is to translate this new findings
into successful therapies.
Strategy
g
To use modern and innovative technologies to further uncover the mechanisms of
paediatric tumour development, progression and relapse. Also, to explore the genetic
and cellular heterogeneity within the same tumour, the regulation of genes (epigenetics),
and the role of the immune system, metabolism and the tumour’s own surroundings
(micro-environment);
4. Develop research in functional imaging, set up a European imaging platform as well as a
platform for quality control in radiation therapy;
5. Improve data sharing, especially those that are linked, such as genomic and clinical data,
and widen access of such information to researchers;
6. Facilitate international academia-led clinical trials (via facilitated submission processes,
shortening the time needed from conception to launch);
7. Widen access to tumour samples and nucleic acids for researchers.
This strategy will be implemented by the ECTGs developing research in each paediatric
malignancy, and it will be facilitated by the cross-tumour European platforms and programmes
set up within SIOPE.
19
A EUROPEAN CANCER PLAN FOR CHILDREN AND ADOLESCENTS
Credit Czech Working Group for Paediatric Oncology,
Czech Republic