Granulosa Theca Cell Tumors of the Ovary

Granulosa theca cell cancers are ovarian tumors that consist of granulosa cells, theca cells, and fibroblasts in various combinations. Granulosa cells in the sex cords produce sex steroids, and peptides needed for folliculogenesis and ovulation. Granulosa cells also give rise to granulosa cell tumors (GCT), which account for approximately 5% of all ovarian neoplasms and 70% of all sex cord-stromal tumors of the ovary. There are 2 distinct types of GCT, adult and juvenile, based on characteristic clinicopathologic features. The adult type is the most typical variety and manifests in peri- or post-menopausal women. In contrast, the juvenile type constitutes only 5% of cases and occurs in prepubertal girls and young women. Theca cells are present in the ovarian stroma and play an essential role in fertility by producing the androgen substrate required for estrogen biosynthesis in the ovaries. Thecomas, comprising less than 7% of sex cord-stromal tumors, are uncommon, usually benign, and have an excellent prognosis. Malignant thecomas are rare and most often also contain an element of granulosa cells, and hence this article focuses on GCTs unless otherwise specified. GCTs are distinct from epithelial ovarian cancers in that they are detected in the early stage, can occur in young females, and usually manifest with abdominal distension, pain, or rarely with features of hyperestrogenism or virilization. Extra-ovarian spread is to the omentum and peritoneum, with occasional hematogenous spread to the lungs, liver, or brain. Lymph node metastases are uncommon. These tumors are treated by surgery alone and have a good prognosis. However, GCTs tend to have an indolent progression prone to late recurrence, seen in up to 25% of cases despite having had curative surgery.