A recent analysis suggests that the use of chemotherapy in the first-line or relapse setting has no impact on survival among patients with sex cord-stromal tumors (SCST). These findings, which were presented during the 2023 ESMO Sarcoma and Rare Cancers Congress, also showed that only surgery-and its quality-led to a progression-free survival benefit for ovarian SCST in the first-line and relapse settings (Abstract 130).
"Ovarian sex cord-stromal tumors are very rare non-epithelial tumors that represent 3-5 percent of ovarian neoplasms," according to study author Helene Vanacker, MD, in the Medical Oncology Department at the Centre Leon Berard in Lyon, France, who noted that it's a "complex histological diagnosis requiring systematic review and potential molecular analysis."
These tumors are classified according to the cell types of origin, she explained. The most common subtypes are adult granulosa cell tumors and Sertoli-Leydig cell tumors, with an age of diagnosis of menopause/perimenopause and young women, respectively.
"Surgery is the cornerstone of treatment," Vanacker noted, "with fertility-sparing surgery for early stage and young patients as compared to radical surgery for advanced disease or FIGO IC2-3-II."
Depending on tumor type and prognostic factors, the postoperative chemotherapy options include bleomycin etoposide carboplatin (BEP) or carboplatin/paclitaxel. In the relapse setting, repeat surgical resections are considered when feasible. There are a number of challenges associated with SCSTs, according to Vanacker. This includes a need for accurate diagnosis, as well as defining prognosis and best standard of care for these patients. The research team initiated the current study to address these gaps in care.
Methods & Results
The researchers collected and analyzed data from 13 TMRG network centers. A total of 469 adult patients with malignant SCST undergoing upfront surgery were enrolled. The inclusion period was from 2011 to July 2015. Vanacker and colleagues performed progression-free and overall survival analyses.
Among enrolled patients, 65.2 percent had early-stage disease (Figo IA-IB), according to the study authors. Stage I disease was reported in 87.5 percent of patients. Stage III and IV disease was seen in 6.7 percent of participants. Seventy-five percent (n=359) of included patients were diagnosed with adult Granulosa cell tumors. A total of 13 percent (n=61) were diagnosed with Sertoli-Leydig cell tumors, and 10.4 percent (n=49) had another rare subtype.
Median follow-up was 6.4 years and 154 (32.8%) patients developed first recurrence during that time, according to the study authors. Of those individuals, second and third recurrences occurred in 82 (17.5%) and 49 (10.4%), respectively.
Vanacker and colleagues reported that 67 (14.7%) patients were administered adjuvant chemotherapy at initial diagnosis. Of those patients, 74.6 percent received BEP and 15.9 percent were administered carboplatin-paclitaxel.
When looking at the relapse setting, data showed that 125 (92.6%), 51 (71.8%), and 25 (59.5%) patients had surgery in the first, second, and third relapse, respectively. Seventy-nine (58.5%), 20 (28.2%), and 10 (23.8%) patients received perioperative chemotherapy in first, second, and third relapse, respectively. Vanacker noted that nine (7%), 12 (17%), and 13 (31%) patients received chemotherapy alone in first, second, and third relapses.
The quality of the surgery was very good, according to Vanacker, with 97 percent of patients undergoing complete (CC0) surgery. Eight (1.7%) patients received incomplete surgery-two patients with Stage II and six patients with Stage III/IV disease.
Survival analyses revealed that first-line therapy, age less than 70 years, FIGO stage, and complete surgery were associated with longer progression-free survival, according to Vanacker and colleagues. Additionally, chemotherapy had no impact on progression-free survival in early-stage disease (FIGO I-II).
The study authors observed a similar progression-free survival when using BEP or other chemotherapy regimens in the first-line setting. However, they reported that at relapse BEP use improved survival when compared with all other chemotherapy regimens. Data revealed a connection between complete surgery and statistically prolonged progression-free survival in the case of recurrence. Vanacker noted that perioperative chemotherapy did not have an impact on progression-free survival.
Study Conclusions
This research sheds light on the role of chemotherapy and surgery among patients with these rare tumors; however, further study is needed to confirm these findings. Vanacker and colleagues underscore the need for randomized clinical trials to confirm that chemotherapy use has no impact on survival in the first-line and relapse setting of sex cord-stromal tumors.
Good quality surgery demonstrates a progression-free survival benefit in the first line, as well as in first and second relapses, Vanacker concluded during her ESMO presentation. However, she noted that, at relapse, incomplete surgery has comparable median progression-free survival to the absence of surgery.
"Chemotherapy for Figo Stage IC and II, at baseline or in relapse after surgery, did not improve progression-free survival," she said. "BEP chemotherapy did not seem to improve efficacy in our cohort compared to other chemotherapy regimens in first-line setting. We need dedicated randomized trials, especially for advanced stage and relapse, to definitely answer the role of chemotherapy after complete surgery."
Catlin Nalley is a contributing writer.