Gonadal Malignancy in Gonadal Dysgenesis: Papillary Pseudomucinous Cystadenocarcinoma in a Patient with Turner's Syndrome

ABSTRACT This report describes a case of papillary pseudomucinous cystadenocarcinoma of the ovary in a patient with Turner's syndrome. This is unique in our experience and in the reported cases in the literature. A survey of the literature suggested that gonadal malignancies occur less often in true Turner's syndrome than in other types of gonadal dysgenesis. Such patients, therefore, should be spared routine prophylactic gonadectomy, particularly in view of their many other problems. The exception is the individual with a hypertrophied clitoris and an XY or XY-mosaic karyotype. Should laparotomy be indicated for any reason in a patient with Turner's syndrome, prophylactic gonadectomy then is indicated.