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3 Cards in this Set

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Peutz-Jeghers Syndrome

Rare autosomal dominant disorder with multiple gastrointestinal hamartomatous polyps and mucocutaneous hyperpigmentation that has am increased risk for developing several malignancies.

What causes Peutz-Jeghers Syndrome

Loss of function mutation in LKB1/STK11 gene which encodes a tumor suppressor protein

Describe the polyps in Peutz-Jeghers Syndrome

Occur in the small intestine and are large pendunculated with a lobulated contour