UTUC is the 3rd most frequent tumour type in the LS spectrum [35] and is particularly common in patients with defects in MSH2, but while European guidelines for LS patient management acknowledge that surveillance for early detection of this tumour is clinically desirable, there are no cost-effective non-invasive techniques routinely available [54]. The gene discussed is MSH2; the disease is renal pelvis/ureter urothelial carcinoma.