Other less frequently encountered hereditary variants impacting pancreatic cancer risk are found in the MMR genes causing LS (4 per cent lifetime risk), PALB2 (2 per cent lifetime risk), TP53 causing LFS (1–2 per cent lifetime risk), CPA1 and CPB1 (0.5 per cent lifetime risk), and other hereditary pancreatitis genes such as CFTR and SPINK-1 (5–20 per cent lifetime risk)178,186,190. Here, TP53 is linked to familial pancreatic carcinoma.