<prompt> True about Peutz jegher syndromeMost common site is small intestinePolypectomy on colonoscopy is treatment of choiceHamaomatous polypAll  <response>: Ans. is a i.e., Most common site is small intestine; b i.e., Polypectomy on colonoscopy is treatment of choice; c i.e., Hamaomatous polyp Peutz -Jeghers syndrome o It is an autosomal dominant condition characterized by :? 1. Hamaomatous polyps in Small intestine -4 100 Colon -- 30 Stomach -- 25 2. Extraintestinal carcinomas of pancreas, breast, ovary, lung and uterus. 3. Mucocutaneous pigmentation o Approximately 3 of patients develop intestinal adenocarcinoma. o The condition is caused by a mutation in the LKB1STK11 gene which codes for a serine - threonine kinase that is thought to play a role in apoptosis. Clinical features o Most common age of involvement is between 20-30 years. o The major symptoms depend on the intestinal location of the polyps i.e. small intestine, colon, stomach :- i Intestinal obstruction intussusception 43 ii Abdominal pain 23 iii Hematochezia 14 iv Prolapse of colonic polyps 7 o There may be mucocutaneous pigmentation :- i Cutaneous pigmentation :- Perioral perinasal region, finger, toes ii Mucous membrane pigmentation :- Buccal mucosa o Other manifestations include precocious pubey, rectal mass rectal polyp, testicular mass, gynecomastia and growth acceleration due to seoli cell tumor. Treatment o Treatment consists of identification of the syndrome and careful monitoring of the tissues that are at risk for cancer development. o Intestinal polyps are usually removed polypectomy when discovered during colonoscopy. 