Dr Mike Murphy & Dr Gerald Draper, University of Oxford

01 January 2011
Second cancers in retinoblastoma patients.

Retinoblastoma is a cancer that affects the lining of the eye (the retina). It affects around 40 children a year in the UK. Almost all patients with retinoblastoma can now be successfully treated, but unfortunately many children with retinoblastoma have a greatly increased risk of developing subsequent cancers. The team wants to better understand the reasons for this increased risk.

Amount of grant: £168,039  |  Date of award: January 2011

Dr Mike Murphy & Dr Gerald Draper, Childhood Cancer Research Group, Oxford

Background
Retinoblastoma accounts for around 3 per cent of all cancers diagnosed in children in the UK.

It is a type of embryonal tumour and, as such, most cases occur in very young children.
Unfortunately many children with retinoblastoma have a greatly increased risk of developing subsequent cancers
Retinoblastoma is one of the few forms of childhood cancer that is known to be caused, in some cases, by an inherited faulty gene. In this case, the faulty gene is the retinoblastoma suppressor gene, RB1.

Around 40 per cent of cases of retinoblastoma are of this ‘heritable’ form and it is these children who have the greatly increased risk of developing other cancers.

Non-ocular cancers associated with retinoblastoma
The Childhood Cancer Research Group (CCRG) has records covering more than 2,000 children diagnosed with retinoblastoma, going back several decades. It is the largest national dataset in the world.

The research team will use these records, together with information on treatment and mutations provided by collaborating centres, to help them better understand why children with inherited retinoblastoma face this greatly increased risk of developing a second cancer.

They are pursuing three avenues of investigation:  

  1. Relation between mutation type and subsequent cancers. Many different types of mutation occur on the retinoblastoma suppressor gene. The team will analyse the types and frequency of subsequent cancers that occur in children with different types of mutation.
  2. Rates of cancers in the parents of retinoblastoma patients. Sometimes one parent of a patient with retinoblastoma will also have retinoblastoma, or they may simply be a carrier of the mutation, without having developed retinoblastoma themselves. The research team will calculate cancer rates in these sub-groups to provide information on risks in later life.
  3. Effects of treatment on risks of subsequent cancers. Children with inherited retinoblastoma appear to be more susceptible to the effects of radiation than other children; it may be that treatment with radiotherapy is responsible for the increased risk of subsequent cancers in these children. The team will attempt to assess the risks of using radiotherapy to treat children with inherited retinoblastoma and will also analyse the possible interactions with chemotherapy and mutation type.

The importance of this research
The analysis of cancer risk in relation to treatment type is of particular importance and will ultimately impact on the way that retinoblastoma is treated in the future.Almost all children diagnosed with retinoblastoma in the UK can now be successfully treated; this means that around 40 children a year are added to the pool of retinoblastoma survivors.

It is of great importance, therefore, to understand the risks that face these children in later life. This study is a major step forward.

The analysis of cancer risk in relation to treatment type is of particular importance and will ultimately impact on the way that retinoblastoma is treated in the future.

One of the reviewers says:
”The applicants, with the extensive series available to them, including complete follow-up are better placed than any other group in the world to address the questions posed.

I recommend funding the proposal. The applicants have a long and productive track record in the field and I am confident that they will deliver on this project.”

Read more: About retinoblastoma


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