The tumour suppressor genes TP53 (Li-Fraumeni syndrome), RB1 (retinoblastoma), NF1 (Von Recklinghausen syndrome), PTCH1 (Gorlin-Goltz syndrome) and TSC1 or TSC2 (tuberous sclerosis) are associated with a very high stochastic risk, but mostly only with a limited increased risk of late effects [20]. Here, PTCH1 is linked to nevoid basal cell carcinoma syndrome.