Sex cord stromal tumors are rare malignant ovarian malignancies that have an overall favorable prognosis and are characterized by early diagnosis, slow growth, and late recurrence. The mainstay of initial management is surgery. No data support postoperative adjuvant treatment for patients with International Federation of Gynecology and Obstetrics (FIGO) stage I, which represent the majority of such tumors. In the last 3 decades, major changes in treatment of sex cord stromal tumors have occurred with the use of more radical surgery, abandonment of radiotherapy, marked decrease in utilization of adjuvant chemotherapy, and the establishment of new chemotherapy regimens, especially those based on platinum. This population-based cohort study was an epidemiologic historic overview that highlighted the importance of FIGO-stage and changes in treatment strategies to improved rates of survival over a study period of 28 years among a population of patients with ovarian sex cord stromal tumors. The study population was comprised of 145 women with a primary sex cord stromal tumor. Data were obtained from the Munich Cancer Registry in Munich, Germany. Two time periods were examined. The first study period was 1978 to 1987 and the second was 1988 to 2005. The Kaplan-Meier method was used to calculate unadjusted estimates of overall survival. Relative survival (an estimate of disease-specific survival) was calculated as the ratio of the observed to expected survival rate. Multivariate analysis with a Cox regression model was used to determine whether age, disease stage (FIGO), postoperative residual tumor, and chemotherapy were independent prognostic factors for infection.
The proportion of women with FIGO-stage I increased from 42.1% in the first study period to 77.8% in the second. Five of 10-year overall survival improved from 55.8%/42.8% (relative survival 58.6%/49.2%) in the first study period to 89.1%/78.3% (relative survival: 92.7%/85.2%) in the second study period. Adjustment for age and FIGO-stage showed that the hazards ratio following surgery in patients with residual tumor was 3.3 (95% confidence interval, 1.5-7.0) compared to those without residual tumor, whereas women without chemotherapy had a hazards ratio of 2.2 (95% confidence interval, 1.2-4.2) in comparison to those with chemotherapy. These findings suggest that the 30% improvement in survival over the 28-year study period result from a stage-shift toward earlier disease stages and advances in treatment such as more radical surgery without residual tumor. Surgery remains the mainstay of treatment. Adjuvant chemotherapy is under active investigation but appears to provide no benefit.