Uterine papillary serous carcinoma in a 32-year-old with Turner's syndrome.

The association of Turner's syndrome and endometrial carcinoma has been previously established but has never been described in conjunction with a uterine papillary serous carcinoma (UPSC). This histological variant is usually found in considerably older women and has no clear relationship to the prior use of estrogen replacement therapy. Despite presenting with stage IV disease, treated by surgery and medroxyprogesterone only, this patient has had an 8-year disease-free remission, suggesting that radical debulking of an endocrine-responsive tumor may be of considerable benefit to some women with this unfavorable histological subtype of endometrial carcinoma.