- Over 90% of granulosa cell tumors have a FOXL2 mutation that contributes to aromatase upregulation.
- Chemotherapy has demonstrated limited efficacy in the treatment of granulosa cell tumors.
- A patient with recurrent granulosa cell tumor responded briefly to anastrazole treatment.
- Retreatment with another aromatase inhibitor letrozole led to a durable response of 24 months.
Granulosa cell tumors (GCTs) represent 3% to 5% of ovarian neoplasms. These tumors arise from the sex-cord stromal cells of the ovary (Colombo et al., 2007). Characteristically, GCTs produce inhibin (Lappöhn et al., 1989) and express aromatase activity promoting estrogen synthesis (Kato et al., 2015). There are two subtypes of GCTs, adult and juvenile, with the former comprising 95% of cases.Melissa Schwartz, Gloria S. Huang,
Most cases of GCT are diagnosed with stage I disease and have a 10-year survival rate of 60–90% (Lauszus et al., 2001). These tumors may recur years to decades after the initial diagnosis, thus necessitating life-long disease surveillance. Cytotoxic chemotherapy and radiation therapy for women with advanced-stage or recurrent disease have shown limited benefit (Schumer and Cannistra, 2003). A systematic review of GCT cases reported in the literature suggested that most respond to hormonal therapy (van Meurs et al., 2014). However, a multi-institutional retrospective cohort study showed only ~ 18% objective response rate among patients with measurable adult GCT treated with hormonal therapy (van Meurs et al., 2015). Although data are sparse, in several reported cases aromatase inhibitorshave been useful in the management of GCT (Freeman and Modesitt, 2006, Lamm et al., 2014). We present herein a unique case of recurrent GCT that previously progressed on the aromatase inhibitor anastrozole but subsequently had a sustained partial response of 24 months duration during re-treatment with another aromatase inhibitor letrozole.
Retreatment with aromatase inhibitor therapy in the management of granulosa cell tumor,
Gynecologic Oncology Reports,