Review Question - QID 216574

This patient with abdominal pain, postmenopausal vaginal bleeding, left adnexal mass, elevated anti-mullerian hormone, elevated inhibin A, and an enlarged endometrial stripe likely has a granulosa cell tumor (GCT). The evaluation of GCTs should include endometrial biopsy in order to guide management.

GCTs are the most common type of gonadal stromal cell tumor, an unusual group of functional (hormone-producing) ovarian tumors. GCTs may secrete large amounts of estrogen that can cause endometrial hyperplasia or carcinoma in older women. Hyperestrogenism is likely in women with a suspected GCT and abnormal uterine bleeding (in all women) or a thickened endometrial stripe (in postmenopausal women only). Endometrial sampling should be performed in all GCT patients with hyperestrogenism in order to guide further management. Younger patients without evidence of endometrial involvement can be treated with unilateral oophorectomy and careful staging in order to preserve fertility. However, in postmenopausal patients or patients with evidence of endometrial involvement, bilateral salpingo-oophorectomy and hysterectomy should be performed.

Schumer et al. review the epidemiology, risk factors, diagnosis, pathologic features, tumor markers, and management of granulosa cell tumors. The authors find that the survival of patients with GCT is generally excellent as most patients present with early-stage disease. However, the authors still recommend prolonged surveillance after treatment with serial physical examination and serum tumor markers (estradiol and inhibin) in patients with GCT.

Incorrect Answers:
Answer 1: Anastrozole is an aromatase inhibitor that suppresses estrogen production from adipose tissues. Anastrozole is used as adjuvant endocrine therapy in postmenopausal patients with hormone-receptor-positive (ER-positive or PR-positive) breast cancer. However, anastrozole would not suppress estrogen production from GCTs.

Answer 3: Neoadjuvant chemotherapy is the use of chemotherapy to reduce the size of a tumor prior to surgery. Neoadjuvant chemotherapy with bevacizumab has been studied in GCT, but it has not been shown to improve outcomes.

Answer 4: Ovarian biopsy is contraindicated when a malignant neoplasm is suspected. Ovarian biopsies may result in the seeding of neoplastic cells along the biopsy tract.

Answer 5: Unilateral salpingo-oophorectomy is an option for treating premenopausal women with GCT. However, in all cases, an endometrial biopsy must be done in order to rule out endometrial hyperplasia or carcinoma which would require a bilateral salpingo-oophorectomy with hysterectomy. In this postmenopausal patient with signs of hyperestrogenism, an endometrial biopsy should be done first.

Bullet Summary:
Endometrial biopsy should be performed in the evaluation of patients with granulosa cell tumors (GCTs) as hyperestrogenism from these tumors can increase the risk of endometrial cancer.