Atlas One · Host Biology · Scale 07 — System

Peutz-Jeghers Syndrome

Peutz-Jeghers syndrome (PJS) is caused by germline STK11/LKB1 mutations; hamartomatous GI polyps + mucocutaneous melanotic spots; cumulative cancer risk by age 70: breast 45%, CRC 39%, pancreatic 36%; intussusception risk; surveillance from age 8.

Also known as: Peutz-Jeghers syndrome, PJS, STK11 hamartoma, LKB1 polyp syndrome, Peutz-Jeghers, hereditary hamartomatous polyposis, PJ polyp, STK11 cancer syndrome, hamartomatous polyposis, Peutz-Jeghers cancer risk

Scale 07 — SystemScale
draftStatus
2026-06-07Last Reviewed
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peutz-jeghers-syndrome

Peutz-Jeghers Syndrome

Peutz-Jeghers syndrome (PJS) is caused by germline STK11/LKB1 mutations; hamartomatous GI polyps + mucocutaneous melanotic spots; cumulative cancer risk by age 70: breast 45%, CRC 39%, pancreatic 36%; intussusception risk; surveillance from age 8.