Professor Jillian Birch, University of Manchester

01 December 2009

International study of non-CNS embryonal tumours – UK pilot

Amount of grant: £324,539

Date of award: December 2009

Professor Jillian Birch, University of Manchester; Professor Patricia McKinney, University of Leeds; Dr Michael Murphy, University of Oxford

This project is part of an international collaboration looking at a group of rare, understudied childhood cancers known as embryonal tumours.

Background

Embryonal tumours occur in many different parts of the body. They are characterised by the proliferation of tissue that is normally only seen in the developing embryo.

This study focuses on those forms of embryonal tumours that develop outside the brain and spinal cord (or central nervous system, CNS); these are known as non-CNS embryonal tumours and include:

These include:

neuroblastoma (sympathetic nervous system)
nephroblastoma/Wilms tumour (kidney)
hepatoblastoma (liver)
retinoblastoma (eye)
and rhabdomyosarcoma (skeletal muscle)

Embryonal tumours are mainly seen in very young children. They account for almost one third of cancer diagnoses in the under fours; or 18% of diagnoses in the 0 to 14 year age group as a whole. On average there are around 230 cases diagnosed annually in England.

The International Study of non-CNS Embryonal Tumours (ISET study)

The relative rarity of non-CNS embryonal tumours makes them difficult to study.

Previous studies have been small and lacking in statistical power and it has not been possible to clarify the role of exposures to suspected risk factors during key periods (preconceptional, prenatal or postnatal).

Opportunities to investigate genetic susceptibility and gene-environment interactions have also been limited.
To overcome these problems, the International Agency for Research on Cancer (IARC) has initiated a multi-centre international study of these tumours.

The study will take place in 15 countries, across five continents, giving access to a much larger number of cases than has been previously available. The larger sample size will address the problems of lack of power encountered in previous studies of these rare tumours.

The full international study will be preceded by pilot studies in participating countries.

UK pilot study

Given the complexity of setting up and conducting such a large project across 15 countries, it is necessary for each participating country to conduct a pilot study before the full-scale study can proceed.

The pilot studies will provide essential information on which to base the full-scale study protocol. They will also allow individual participating centres to identify and resolve issues and problems specific to their country.

To quote one of the reviewers:
“This is a superbly qualified team with a long track record of working together and of working with all the critical portions of the international collaboration that underlies the overall project. The proposed centres are the very best that can be envisioned and are fully capable of supporting the proposed work.”

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