- -Hereditary Intestinal Polyposis
- nautosomal dominant condition characterized by:
- multiple hamartomatous polyps of the gastrointestinal
- tract - most often in the small bowel but may occur affect any portion of the GI tract
mucocutaneous pigmentation - mainly, ofthe lips, buccal mucosa, genitalia, hands and feet
- Patients often present with small bowel
- intussusceptions 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.