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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.



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


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.



Credit Czech Working Group for Paediatric Oncology,

Czech Republic