Fewer than 5% of ovarian malignancies are sex cord stromal tumors (SCST), the majority of which are granulosa-cell tumors. Other types include Sertoli-Leydig cell neoplasms and theca-cell tumors. SCST are found in younger women than are epithelial ovarian tumors, and they generally follow an indolent course. The investigators attempted to identify clinical and pathological prognostic factors affecting the outcome of SCST in 376 women with a median age of 51 years who presented with granulosa-cell tumors (n = 339) or Sertoli-Leydig cell tumors (n = 37). More than two thirds of women (71%) had stage I, 10% stage II, 11% stage III, and 8% stage IV disease at the time of presentation. Advanced-grade disease was present in 39% of 106 women whose tumor grade was known.
Overall 5- and 10-year disease-specific survival rates were 88% and 79%, respectively. Patients with early-stage (stage I-II) disease had survival rates of 95% at 5 years and 84% at 10 years. The corresponding figures for women having advanced-stage (stage III-IV) disease were 59% and 57% (P < 0.001). Women aged 50 years or less had a 5-year survival advantage over their older peers (93% versus 84%, P < .001). Patients with lower-grade tumors had better 5-year survival than those with grade 3 lesions (96% versus 64%, P < 0.001). Tumor size was not an important predictor of survival. The 134 women aged 50 or younger who had stage I disease had a 10-year survival rate of 94%. No survival difference was evident between women having standard or uterine-sparing surgery. On multivariate analysis, factors predicting better survival included younger age at diagnosis and early-stage disease.
This series—the largest yet reported of women with SCST of the ovary—identifies relatively young age and early-stage disease at diagnosis as significant predictors of longer disease-specific survival. The findings also indicate that conservative surgery is a safe option for women with early-stage disease who wish to remain fertile.