PJS is a rare autosomal dominant inherited disorder caused by a germline PV in the STK11 gene, also known as the LKB1, which is located on 19p13.3 and comprises nine coding exons and one noncoding exon, coding a member of the serine/threonine kinase family with 433 amino acids. This evidence concerns the gene STK11 and Peutz-Jeghers syndrome.