From the Guidelines
The primary treatment for sex cord-stromal tumors (SCSTs) involves surgery, with adjuvant therapies considered based on tumor type, stage, and patient factors, and platinum-based chemotherapy regimens such as BEP are commonly used for advanced or recurrent disease 1.
Treatment Approach
The treatment approach for SCSTs is multifaceted and depends on several factors including the stage and type of tumor, as well as the patient's overall health and reproductive desires.
- For early-stage SCSTs, surgery alone may be sufficient, especially for stage IA granulosa cell tumors, which have an excellent prognosis after surgical resection alone and do not require adjuvant therapy 1.
- For advanced or recurrent disease, platinum-based chemotherapy regimens are the mainstay of treatment, with the BEP combination (bleomycin, etoposide, and cisplatin) being a commonly used regimen 1.
- Alternative chemotherapy options include paclitaxel and carboplatin, etoposide plus cisplatin, and cyclophosphamide, doxorubicin, and cisplatin 1.
Patient Considerations
Patient factors, such as age and pre-existing medical conditions, play a significant role in treatment decisions.
- For example, bleomycin should not be given to patients over 40 years old or those with pre-existing pulmonary disease due to its potential toxicity 1.
- Hormone therapy may be beneficial for patients with hormone-producing tumors, with options including leuprolide acetate, megestrol acetate, or aromatase inhibitors like letrozole 1.
Ongoing Research
Ongoing research is exploring new treatment options for SCSTs, including the use of anti-angiogenic agents like bevacizumab, which has shown promise in preliminary studies 1.
- The GINECO group is currently conducting a phase II trial to explore the clinical benefit of adding bevacizumab to weekly paclitaxel followed by bevacizumab as maintenance versus weekly paclitaxel followed by observation in patients with relapsed SCSTs 1.
From the Research
Treatment Overview
The treatment for sex cord-stromal tumors (SCST) typically involves surgery, with the goal of removing the tumor and preserving fertility when possible.
- Surgery is the primary treatment for SCST, with the type of surgery depending on the stage and location of the tumor, as well as the patient's age and fertility desires 2, 3, 4, 5, 6.
- Inguinal orchiectomy and testis-sparing surgery are recommended for certain conditions, while retroperitoneal lymph node dissection is used as a staging tool to determine the follow-up protocol 2.
Surgical Options
Different surgical options are available for SCST, including:
- Fertility-sparing surgery (FSS), which includes cystectomy, unilateral salpingo-oophorectomy (USO), or bilateral salpingo-oophorectomy (BSO) with uterine preservation 6.
- Total hysterectomy with unilateral or bilateral salpingo-oophorectomy (TAH-USO/BSO) 6.
- Complete staging surgery (CSS), which includes peritoneal cytologic examinations, inspections of peritoneal surfaces, random peritoneal biopsies, and omentectomy 6.
- Debulking surgery, which is used to remove as much of the tumor as possible 6.
Chemotherapy and Hormonal Therapy
Chemotherapy and hormonal therapy may be used in some cases, particularly for patients with advanced or recurrent disease.
- Platinum-based chemotherapy is currently used for patients with advanced stage SCSTs or recurrent disease, with an overall response rate of 63% to 80% 3.
- Hormonal or targeted therapy may be attempted on an individualized basis in cases where chemotherapy fails 4.
Follow-up and Prognosis
Follow-up is essential for patients with SCST, as the disease can recur late and have an indolent clinical course.
- The first 2 years are important in the follow-up of the disease, and the follow-up protocol can be determined individually according to the aggressive properties of the disease and patient status 2.
- Patients with malignant clinicopathologic features and metastatic disease may require more vigorous follow-up 2.