B122
Familial cancer risk in a cohort of children with Wilms tumour or rhabdomyosarcoma
Robert Alston, Marco Geraci, Ramandeep Arora, Tim Eden, Jillian Birch
University of Manchester, UK
Background
For some childhood tumours there are well documented associations with congenital anomalies, extreme birth weights and familial cancer history. Less well known is the possible relationship between the familial cancer risks, birth weight and congenital anomalies in the cases.
Method
Data on all children under 15 years of age with Wilms tumour (325 cases) or rhabdomyosarcoma (218 cases) registered with the Manchester Childrens Tumour Registry (MCTR) from 1954 to 2008 and those enrolled in the population-based UK Childrens Cancer Study (UKCCS) from Merseyside and North Wales from 1992 to 1996 were included. Information on birth weights and congenital anomalies in the index cases was available. Medical histories of index cases first and second degree relatives and first cousins were collected in 97% of families. First degree relatives were flagged for cancer notifications and death. Cancer risks in relatives of the index cases were analysed.
Results
For relatives of cases with Wilms tumours, significant increased risks of cancer were found for retinoblastomas (relative risk = 18.4) and soft tissue sarcomas (STS, RR = 2.9). These increases were limited to relatives of index cases whose birth weight was between the 10th and the 90th centile of the population distribution and to relatives of index cases with at least one congenital anomaly. For relatives of rhabdomyosarcoma index cases, there were increased risks of neuroblastomas (RR = 7.9), osteosarcomas (RR=11.1) and chondrosarcomas (RR=16.52). For those with at least one congenital anomaly, there was an increased risk of STS (RR=5.61).
Conclusion
These results suggest that there may be links between germline mutations in oncogenes, increased familial cancer risk and congenital anomalies for families of both Wilms tumour cases and rhabdomyosarcoma cases.
