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Chapter 4: Gastrointestinal oncology 183

Complications Aetiology
Malignantchangeisinevitableaseachpolypcarriesarisk Autosomal dominant inheritance pattern, most cases in-
of transformation. Adenocarcinoma is unlikely before volvemutations in the PJ gene on chromosome 19.
the age of 20.
Clinical features
Investigations Patients are found to have mucocutaneous pigmenta-
Colonoscopy is used to screen relatives above 12 years. tioncharacteristicallyaroundthemouth,handsandfeet.
Gastrointestinal hamartomatous polyps are found in the
Management
small bowel, colon and stomach.
Definitive treatment involves a total colectomy and ileo-
rectalanastomosiswithilealpouchformation.Therectal
Complications
stump requires continuing surveillance.
Polyps may bleed or cause intussusception, but are not
pre-malignant.
Peutz–Jegher syndrome
Definition Management
Syndrome characterised by intestinal polyposis and Multiple polypectomies may be required, but bowel re-
freckling of the lips. section should be avoided.