Our Researcher in Focus for September is Dr. Noelle Cullinan. Noelle is a Clinical Research Fellow in the Department of Paediatric Haematology & Oncology at Children’s Health Ireland, Crumlin, and is a National Children’s Research Centre Clinical Research Fellowship Awardee (2019-2021).
Under the supervision of Prof. Cormac Owens and Prof. Owen Smith, Noelle is completing a Masters Degree through University College Dublin with a project entitled “Exploring Cancer Predisposition Syndromes and the Risk of Developing Secondary Malignancies in the Irish Paediatric Oncology Population”.
In exploring the background to her project, Noelle explains “Between 150 and 200 children (aged 0-16 years) are diagnosed with cancer each year in Ireland. With appropriate treatments such as surgery, chemotherapy, radiation therapy, and more recently, immunotherapy, over 80% of children now survive their cancer”.
Treatments for cancer are, however, not without their complications. “Many childhood cancer survivors develop long-term complications of their disease and its therapy, and a small number of survivors go on to develop subsequent cancers” explained Noelle.
Although this process is complex, Noelle explains further “Risk factors for developing subsequent cancers include exposure to chemotherapy and radiation, with genetic predisposition increasingly implicated. We now know that at least 10% of children with cancer have an underlying cancer predisposition syndrome. These children may develop multiple cancers over a lifetime, and identification of a cancer predisposition syndrome can have implications, not just for the affected child, but also potentially other family members”.
If clinicians were able to identify children with cancer who are genetically predisposed to cancer development, they could initiate close monitoring in the hope of identifying subsequent cancers earlier, ultimately leading to better outcomes. “Children diagnosed with an underlying cancer predisposition syndrome may be eligible for disease surveillance strategies and enhanced follow-up to monitor for subsequent cancer development. Early detection of secondary cancers can in turn lead to diagnosis of tumours at a lower stage, potentially requiring reduced treatment intensity, and resulting in better overall outcomes for patients. This has an impact for cancer survivors of all ages” explained Noelle.
In Ireland, there is currently limited data on the incidence of and risk factors for the development of second cancers, which prompted Noelle to address this in one aspect of her research project “This study aims to describe the Irish childhood cancer survivor population and quantify the occurrence of subsequent malignancies in survivors”. National cancer registry data is being interrogated to describe the incidence, timing and features of primary and subsequent cancers, and relevant treatment exposures, in children and young adults (0-19 years) who were diagnosed with their first cancer in the years 1995 – 2014“.
Noelle is also exploring the feasibility of using clinical risk prediction tools in children with cancer in order to prioritise and streamline referrals for genetic evaluation. These clinical risk prediction tools include the McGill Interactive Pediatric OncoGenetic Guidelines (MIPOGG), that Noelle helped to develop during her oncology fellowship at The Hospital for Sick Children (SickKids) in Toronto.
Her recent publication in the Journal of Clinical Oncology shows that application of the MIPOGG tool in childhood cancer survivors can help to predict which cancer survivors are at highest risk of developing subsequent cancers in follow-up.
Ultimately, it is hoped that Noelle’s study will lead to improvements in the treatment and follow-up of childhood cancer survivors in Ireland. “This study will generate useful data for the Irish childhood cancer survivor population and will be valuable in allowing us to plan cancer surveillance strategies for survivors, while also working towards the development of a dedicated cancer genetic services for the paediatric oncology population” said Noelle.
More information on Noelle’s research can be found here:
Utility of a Cancer Predisposition Screening Tool for Predicting Subsequent Malignant Neoplasms in Childhood Cancer Survivors. Noelle Cullinan et al. Journal of Clinical Oncology. DOI: 10.1200/JCO.21.00018
McGill Interactive Pediatric OncoGenetic Guidelines app website: MIPOGG