Peutz-Jegher's syndrome is an autosomal dominant condition characterised by:
Patients often present with small bowel intussusception before the age of 10 years.
The polyps themselves have a very low malignant potential. About 10-20% of patients develop gastrointestinal carcinoma but this is thought to arise from coexistent adenomas.
Patients have an increased risk of developing carcinomas of the pancreas, lung, ovary and breast.
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