Thematic disease groups
A genetic tumour risk syndrome (genturis) is a genetic disorder in which inherited genetic mutations in one or more genes strongly predispose the individuals to the development of benign or malignant tumours, sometimes with childhood onset. Most patients develop multiple primary tumours in various organ systems. The lifetime risk for cancer can be as high as 100%.
The syndromes are grouped in four thematic groups that have overlap in manifestations and/ or genetic cause and a group of even more rare syndromes with predominantly malignant tumours.