- AD; mutation in STK11/LNK1 gene
- Tumor supressor gene that controls the development of pathogenic precursor hamartomas of adenocarcinomas
- Pigmented macules on lips, buccal mucosa, digits
- Present by age 20
- Can fade with time
- GI polyps
- Intussusception
- GI bleeds, anemia
- Vomiting
- Malignancy
- Breast (50%), GI (40%), pancreatic (35-40%)
- Also ovarian and cervical
- Adenocarcinoma in younger patients
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