Cause:Neoplasia; idiopathic and perhaps radiation-induced

Pathophys:Arises from cortical stroma or wall of involuting follicle, especially in aging ovaries. It is curious that it presents as an estrogen-producing tumor, yet granulosa cells or corpus luteum produce mainly progesterone. Estrogen production is never more than that during a normal menstrual cycle

In women and girls; 60% are postmenopausal; 10% of all ovarian cancers (Nejm 1989;321:790); incidence is 1/100 000 women age >19 yr; 1/54 000 women age 50-65. The only pediatric functional ovarian tumor

Sx: Abdominal pain, tumor; secondary amenorrhea and menometrorrhagia. Precocious puberty (vaginal bleeding), r/o trauma/abuse, exogenous estrogen, craniopharyngioma, luteoma

Si:Abdominal mass

High survivals; can recur decades later; benign in children except for the precocious puberty

Meigs' syndrome (ascites and hydrothorax) with benign types as well as more common benign fibromas of ovary

Malignancy, in <40%; endometrial carcinoma, especially in those over age 50

Lab:

Chem:Inhibin levels correlate with tumor presence and bulk (Nejm 1989;321:790)

Path:12% are microscopically bilateral

Rx:Surgery; radiation is very good for recurrences