title: Endometrial adenocarcinoma without prior hormone replacement in a diabetic patient with gonadal dysgenesis.
abstract: Patients with dysgenetic gonads and Turner syndrome are unlikely to develop endometrial carcinoma unless they have received unopposed estrogen replacement therapy. This case describes a 54-year-old woman with Turner syndrome and primary amenorrhea who developed adenocarcinoma of the endometrium without having received hormone replacement. Vaginal bleeding, a pelvic mass, and sepsis were the presenting symptoms. The patient also had diabetes mellitus and hypothyroidism. Polyglandular endocrine patterns are known to occur with a high frequency in these patients. The woman's chromosome studies revealed a modified 46,X,i(Xq) (isochromosome X). This is the first report of an isochromosome X patient to develop endometrial cancer without receiving estrogen replacement. The etiology of this rare case may be an increased propensity for patients with X-chromosome deletions to develop neoplasms in general, or extragonadal estrogen production.
authors.affiliation: Department of Obstetrics and Gynecology, State University of New York Health Science Center, Syracuse.
authors.forename: K
authors.initials: K
authors.lastname: Kratzert-Adams
authors.forename: M D
authors.initials: MD
authors.lastname: Adelson
authors.forename: S Z
authors.initials: SZ
authors.lastname: Badawy
authors.forename: C K
authors.initials: CK
authors.lastname: Stein
authors.forename: L
authors.initials: L
authors.lastname: Gordon
mesh_terms: D000230:Adenocarcinoma / Q000150:complications*; D003924:Diabetes Mellitus, Type 2 / Q000150:complications*; D016889:Endometrial Neoplasms / Q000150:complications*; D015914:Estrogen Replacement Therapy / Q000009:adverse effects; D005260:Female; D006801:Humans; D008875:Middle Aged; D012307:Risk Factors; D014424:Turner Syndrome / Q000150:complications*