Answer: Pop Quiz #9

Correct Answer:  2. Follicular Thyroid Cancer

Explanation:  The flesh-colored facial papules pictured here are trichilemmomas of Cowden Syndrome.  Cowden Syndrome is an autosomal dominant mutation in the PTEN tumor suppressor gene.  Other mucocutaneous findings include oral papillomas, acral keratoses, and sclerotic fibromas.  Patients are at an increased risk for thyroid cancer (follicular most common), fibrocystic breast disease, breast cancer, GI polyps, ovarian cysts, and uterine leiomyomas.  Renal cell carcinoma would occur at a higher rate in patients with Birt-Hogg-Dube Syndrome.  Pheochromocytoma occurs in patients with MEN2A or MEN2B and neurofibromatosis.  Medulloblastoma may occur in patients with Gorlin Syndrome.

Explanation courtesy of Jordan Heskett, MD

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