Granulosa Cell Tumors

Introduction

• Description: A granulosa cell tumor is a sex cord stromal tumor of the ovary made up of granulosa cells (sex cord) and stromal cells (thecal cells or fibroblasts). The tumor often secretes estrogen.

• Prevalence: 2%–5% of ovarian neoplasms and the majority of hormonally active tumors. Absolute rate of less than 0.2/100,000 women.

• Predominant Age: Any; 5% before puberty; most before the age of 40 years.

• Genetics: No genetic pattern. Somatic mutations in FOXL2 have been identified in 97% of adult subtype granulosa cell tumors. No association with the BRCA germline mutations.

Etiology and Pathogenesis

• Causes: Unknown.

• Risk Factors: Obesity, family history of breast or ovarian cancer, non-White race. Oral contraception, parity, and smoking reduce the risk.

Signs and Symptoms

• Asymptomatic.

• Enlarging or ruptured adnexal mass (may present with acute pain and an acute abdomen with hemoperitoneum, 6%); 10%–15% are not palpable; tumors are bilateral in less than 2% of cases; average size at diagnosis is 12 cm.

• Ascites (10%).

• Precocious (pseudoprecocious) puberty in young children (5%; granulosa tumors are responsible for 10% of precocious puberty cases).

• Abnormal menstrual patterns, menorrhagia, amenorrhea.

• Postmenopausal bleeding.

Diagnostic Approach