By Disease Name > Cowden's syndrome
Cowden's syndrome
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AKA multiple hamartoma syndrome
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pathogenesis
–
PTEN gene;
autosomal dominant
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= multiple hamartomas and increased risk of breast and thyroid cancer
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pathognomonic hamartoma =
tricholemmomas
(on central face)
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majority of tumors (including
breast and thyroid
(e.g. fibrocystic disease of the breast and thyroid adenomas)
) benign
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10% develop non-medullary thyroid CA
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50% of affected females develop breast CA