"Average age of first diagnosis 23.
Features:
- Hamartomatous polyps in small intestine
colon
bronchus
nose
- Hyperpigmented maccules on the lips
palms and soles.
- Bowel obstruction from intussusception.
- GI bleed
The polyps only have a small malignant potential.
However there is an Increased risk of carcinoma of :
GI tract: oesophagus
stomach
small bowel
colon
rectum
Pancreas
liver
lungs
Breast
Ovaries.
Thyroid
50% of patients die from cancer by the age 57.
Cancer susceptibility arises from mutation activating serine/threonine kinase."