Symptoms of Peutz Jeghers syndrome
- This autosomal dominant trait is often heralded by the presence of melanin spots on the lips and buccal mucosa.
- Hamartomas are almost always present on the small intestine and occasionally on the stomach and colon.
- Previously considered a benign process, patients with PJS are at increased risk for multiple cancers: breast (50%), GI (50%), pancreatic (35%), gynecologic (10%-20%), and testicular (< 10%) .
- Aggressive cancer screening programs are recommended for persons with PJS.
