Cowden syndrome (CS) is a rare autosomal dominant disorder demonstrating variable penetrance and an incidence of 1:200 000. The syndrome is characterized by gastrointestinal polyposis, mucocutaneous hamartomas of face and oral papillomatosis. Eighty percent of patients with CS have mutation in phosphatase and tensin homolog tumor-suppressor gene making them prone for developing malignancies of breast, thyroid and genitourinary system. Arteriovenous malformations (AVM) in brain, vertebral column and skin are also common features [1].
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