Treatment patterns and survival outcomes of recurrent adult-type granulosa cell tumors

Abstract

Objectives: Granulosa cell tumors are characterized by indolent growth and late recurrence; treatment of recurrent disease is not standardized. We sought to evaluate the outcomes of patients who underwent secondary and tertiary cytoreductive surgery as part of treatment for recurrent granulosa cell tumor. Methods: A retrospective review was conducted of women diagnosed with primary or recurrent granulosa cell tumors at an urban university system between 2010 and 2019. Data collected included demographic, clinicopathologic, and treatment factors. Endpoints of treatment outcome was progression free survival. Statistical analyses were preformed using the Kaplan-Meier method, the log-rank test, and Fisher's exact test. Results: 75 patients diagnosed with primary or recurrent granulosa cell tumor were identified. After excluding cases with inadequate follow-up and juvenile granulosa cell tumor, 69 patients were analyzed. The average age at diagnosis was 48.3 years (21.2-86.0). 82.6% of patients had stage I disease; the remainder had stage II (8.7%) or stage III (8.7%) disease. 15 of 69 patients (21.7%) were treated for recurrent disease. The average time to recurrence was 6.27 years (range 1.05-20.71). There was a significant correlation between optimal resection or staging (including omentectomy and peritoneal biopsies) and recurrence (OR 0.171, 95% CI 0.0553-0.560, p=0.0076). Fertility preservation was not associated with recurrence (p=0.08). 71% of women with recurrent disease underwent secondary cytoreduction. Secondary CRS was associated with a significantly higher progression free survival compared to medical management alone at time of recurrence (median 2.3 vs 1.6 yrs, p=-0.01). 10 women were subsequently treated for a second recurrence; 50% were treated with cytoreductive surgery. No PFS benefit was seen with surgical management compared to medical management for the second recurrence (p=0.31). Seven women were then treated for a third recurrence or progression and six women for a fourth. PFS after treatment for second, third, or fourth recurrence or progression was longer among patients whose treatment included surgery compared to those who did not (median 1.708 vs 0.997 years), although this difference was not statistically significant. Conclusions: Complete staging for granulosa cell tumors was associated with decreased risk of recurrence. Secondary CRS at first recurrence is associated with a significantly higher progression free survival.