**Question:** A 32-year-old man presents with abdominal pain. On examination, he is noted to have pigmented spots in the buccal region. He is diagnosed to have Peutz-Jeghers syndrome, which also results in what? SELECT ONE.

A. Melanosis coli
B. Hamartomatous polyps in the gastrointestinal tract
C. Pulmonary artery hypertension
D. Breast cancer

**Correct Answer:** .

**Core Concept:** Peutz-Jeghers syndrome is an autosomal dominant disorder characterized by the presence of mucocutaneous mucus stains (pigmented spots) and hamartomatous polyps in the gastrointestinal tract.

**Why the Correct Answer is Right:** Peutz-Jeghers syndrome is a rare genetic disorder caused by mutations in the LAMB1, STK11, or TP53 genes. This condition is characterized by mucocutaneous mucus stains (pigmented spots) and hamartomatous polyps in the gastrointestinal tract, which can lead to gastrointestinal bleeding, obstruction, and even cancer.

**Why Each Wrong Option is Incorrect:**

A. Melanosis coli is a condition characterized by hyperpigmentation of the colonic mucosa, not commonly associated with Peutz-Jeghers syndrome.
B. While gastrointestinal polyps are a common feature of Peutz-Jeghers syndrome, the correct answer is hamartomatous polyps instead of "Hamartomatous polyps in the gastrointestinal tract."
C. Pulmonary artery hypertension is not a feature of Peutz-Jeghers syndrome, unlike the correct answer of gastrointestinal involvement.
D. Peutz-Jeghers syndrome is not associated with an increased risk for breast cancer, unlike the correct answer of gastrointestinal involvement.

**Clinical Pearl:** Peutz-Jeghers syndrome is an important differential diagnosis to consider in patients presenting with mucocutaneous mucus stains and gastrointestinal symptoms, particularly in the presence of a positive family history. Early diagnosis and surveillance are crucial to prevent complications like gastrointestinal bleeding, obstruction, and cancer.