Cowden's disease

Cowden syndrome

A rare autosomal dominant genodermatosis (OMIM:158350) characterised by multiple hamartomas arising from all three germ cell layers, most prominently from the ectoderm and endoderm, in particular hamartomas of skin and the GI tract (from the stomach to the colon) and an increased risk of cancer (e.g., breast, thyroid (papillary or follicular), uterus and osteosarcoma). Women with Cowden syndrome have up to a 50% lifetime risk of developing breast cancer; 75% have benign breast disease (fibroadenomas, fibrocystic changes).
A rare autosomal dominant hamartoma/neoplasia syndrome [MIM 158350] characterised by multiple hamartomas—which arise from all three germ cell layers, most prominently from the ectoderm and endoderm, in particular hamartomas of skin and the GI tract, from the stomach to the colon—and an increased risk of developing cancer, including the breast, thyroid, and uterus. Women with Cowden’s syndrome have up to a 50% lifetime risk of developing breast cancer and up to 75% have benign breast disease—fibroadenomas, fibrocystic changes
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References in periodicals archive ?
Winans, "Ganglioneuromatosis of the colon and extensive glycogenic acanthosis in Cowden's disease," Digestive Diseases and Sciences, vol.
ANTONIA COWDEN'S DISEASE WHEN Antonia Palmer suffered a series of agonising headaches, she opted to grin and bear it.
It wasn't until the age of 34 that she learned she had Cowden's disease - a rare illness which puts her a higher risk of cancer.
Up to 50% of women with Cowden's disease get breast cancer, and 10% are diagnosed with thyroid cancer, he added.
Up to 50% of women with Cowden's disease get breast cancer, and about 10% are diagnosed with thyroid cancer, he added.
This disease is often associated with a familial (autosomal dominant) hamartoma-neoplasia syndrome called Cowden's disease (CD).
Cowden's disease is a hereditary disorder characterized by oropharyngeal fibrosis and multiple hamartomas with potential malignant changes.
Cowden's disease (multiple hamartoma syndrome), first described in 1963 by Lloyd and Dennis, is a rare autosomal-dominant disease.
To our knowledge, there has been no previous report in the literature of Cowden's disease in which the fibrotic lesions affected a vocal fold.
In view of the constellation of clinical features--oropharyngeal fibrosis and papillary lesions on the pharynx, larynx, lips, left vocal fold, and skin-a diagnosis of Cowden's disease was made.
* Cowden's disease. This condition, also known as multiple hamartoma-neoplasia syndrome, causes hamartomas that involve the skin, intestine, breast, and thyroid.