The first is Peutz‐Jeghers Syndrome, which is caused by germline mutations in the STK11/LKB1 tumor suppressor gene [relative risk (RR) = 132, cumulative risk from age 15 to 64 years = 36%]6; the BRCA2 gene mutation (10‐fold higher risk for PC than the general population)7, 8, 9; the CDKN2A gene mutation associated with the Familial Atypical Multiple Mole Melanoma (FAMMM) Syndrome (16% lifetime risk of PC)10; and hereditary pancreatitis mostly associated with the SPINK1 gene mutation (cumulative risk of PC of 8–11% at the age of 50 years).11 This evidence concerns the gene SPINK1 and pachyonychia congenita.