Adult
granulosa cell tumor (
GCT) of the ovary is oftentimes a hormonally active, stromal cell
neoplasm that is distinguished by its ability to secrete sex
steroids such as
estrogen. Patients may present with
vaginal bleeding caused by
endometrial hyperplasia or
uterine cancer as a result of prolonged exposure to
tumor-derived
estrogen. In addition, GCT is a vascular
tumor that may occasionally
rupture and result in
abdominal pain,
hemoperitoneum, and
hypotension, mimicking an
ectopic pregnancy in younger patients. GCT is usually associated with a mass on pelvic examination that is subsequently confirmed on ultrasonography. Surgery is required for definitive tissue diagnosis, staging, and
tumor debulking. In older women, a total abdominal
hysterectomy and bilateral salpingooophorectomy are typically performed. In women of childbearing age, a more conservative unilateral
salpingo-oophorectomy may be performed, assuming that careful staging reveals that the disease has not extended outside of the involved ovary and that a concomitant
uterine cancer has been excluded. Survival of patients with GCT is generally excellent because most patients present with early-stage disease, although certain high-risk patient groups may be identified. Stage is the most important prognostic factor, with a higher risk of relapse being associated with stages II through IV disease. In addition, patients with stage I disease associated with features such as large
tumor size, high mitotic index, or
tumor rupture may also be at higher risk in some series. The value of postoperative adjuvant
therapy for high-risk patients has not been investigated by prospective randomized trials, which are difficult to perform because of the rarity of this
tumor. Nonetheless, the use of
adjuvant chemotherapy or radiation has sometimes been associated with prolonged disease-free survival in patients with high-risk features. Because of the propensity of GCT to recur years after initial diagnosis, prolonged surveillance with serial physical examination and serum
tumor markers such as
estradiol and
inhibin is reasonable.