peutz-jeghers syndrome with diffuse gastrointestinal polyposis: three cases in a family with different manifestations and no evidence of malignancy during 14 years follow up

Peutz-Jeghers Syndrome With Diffuse Gastrointestinal Polyposis: Three Cases in a Family With Different Manifestations and No Evidence of Malignancy During 14 Years Follow Up.

INTRODUCTION Peutz-Jeghers syndrome (PJS) is a rare disorder characterized by mucocutaneous perioral pigmentation, gastrointestinal hamartomatous polyposis, and an increased risk of malignancy. Families with PJS may show a variable spectrum of manifestations in spite of their consecutive generations. A probable explanation is novel mutations in contributing genes. CASE PRESENTATION This repor...

Peutz - Jeghers syndrome associated with gastrointestinal carcinoma

Patients with the Peutz-Jeghers syndrome carry a slight, though definite, increased risk of gastrointestinal carcinoma.The malignant potentiality of Peutz-Jeghers hamartomatous polyps, generally considered benign, is supported by this report. Two cases of metastasising gastrointestinal carcinoma associated with the Peutz-Jeghers syndrome are described in a 56 year old female and her 29 year old...

Unusual manifestations of the Peutz-Jeghers syndrome.

Seven-Year Follow-Up of Peutz-Jeghers Syndrome

One of the clinicopathological criteria for diagnosing Peutz-Jeghers syndrome (PJS) is mucocutaneous pigmentation. We present a 57-year-old Iranian female patient with diffuse pigmentation in buccal and labial mucosa. The first colonoscopy revealed one 0.5 cm rectal polyp. However surveillance colonoscopies over a 7-year polyp showed over 100 colorectal polyps.

A case with Some Components of Cronkhite-Canada Syndrome in a Family with Peutz-Jeghers Syndrome

Peutz Jeghers syndrome (gastro-intestinal polyposis) and its complications.

A rare case of multiple jejunal poiyps, presenting as an acute abdominal emergency with complications of mtussusception and gangrene is discussed. The relevant features of Peutz Jegher’s syndrome and the surgical management of it’s complications are highlighted (JPMA 35 : 154, 1985).