Management of reproductive health in Cowden syndrome complicated by endometrial polyps and breast cancer.

[cowden syndrome]

Cowden syndrome is an autosomal-dominant condition associated with mutations in the tumor suppressor gene PTEN . Gynecologic malignancies are common with a 5 -10 % risk of endometrial cancer and 25 -50 % risk of breast cancer .A 37 -year -old woman with a history of breast cancer , other neoplasms , and multiple skin lesions was diagnosed with Cowden syndrome after a germline PTEN mutation was identified . The endometrium had high glucose uptake on positron emission tomography scan and was irregularly thickened on ultrasonography ; biopsy revealed endometrial polyps and simple hyperplasia . Fifteen months later , hysteroscopy again confirmed numerous benign endometrial polyps .Recurrent , multiple endometrial polyps portend a high risk of endometrial cancer in women with Cowden syndrome . Monitoring for malignancy and consideration of hysterectomy after childbearing is completed is warranted .