28 March 1997

I have an 11y child with onset of severe desmoid disease in the shoulder region. She has a 2nd degree relative with Colorectal cancer at 60 years of age. No family history of desmoids, sebaceous cysts or adenomas. In view of the recent identification of the APC mutations in Hereditary Desmoid disease and the few case reports of familial disease what is the risk to subsequent children? Mother and father have no clinical disease but have not had sigmoidoscopy. Initial thoughts were that the risk was negligible. Has that changed?

Thanks