From the Guidelines
Surgical resection is the primary treatment for sex cord tumors, with the extent of surgery depending on tumor size, stage, and patient's desire for fertility preservation, and chemotherapy regimens such as BEP or paclitaxel and carboplatin may be considered for advanced or recurrent disease. The management of sex cord tumors involves a multidisciplinary approach, taking into account the tumor's histology, stage, and the patient's overall health and fertility desires.
Key Considerations
- For ovarian sex cord tumors, unilateral salpingo-oophorectomy may be sufficient for early-stage disease in younger patients, while complete hysterectomy with bilateral salpingo-oophorectomy is often recommended for advanced disease or postmenopausal women 1.
- For testicular sex cord tumors, radical orchiectomy is the standard approach.
- Chemotherapy regimens using bleomycin, etoposide, and cisplatin (BEP) or paclitaxel and carboplatin may be considered for advanced or recurrent disease, with a response rate of 63%–80% reported for platinum-based chemotherapy in patients with advanced-stage sex cord-stromal tumors (SCSTs) 1.
Follow-up and Surveillance
- Long-term surveillance is essential as these tumors can recur years after initial treatment, with recommended follow-up including physical examinations, tumor marker assessments (inhibin, estradiol, testosterone), and imaging studies every 3-6 months initially, then annually.
- The use of hormone therapy, such as gonadotropin-releasing hormone (GnRH) agonists, tamoxifen, progestin, and aromatase inhibitors (AIs), may be considered for patients with advanced-stage or recurrent granulosa cell tumors, with a reported response rate of 25.8% complete response and 45.2% partial response 1.
Recent Guidelines and Recommendations
- The European Society for Medical Oncology (ESMO) recommends debulking surgery, whenever feasible, as the most effective treatment for metastatic or recurrent granulosa cell tumors, with platinum-based chemotherapy used for patients with advanced-stage SCSTs or recurrent disease 1.
- The National Comprehensive Cancer Network (NCCN) guidelines recommend fertility-sparing surgery for patients with stage IA or IC sex cord-stromal tumors who desire to preserve their fertility, with postoperative options including observation or consideration of platinum-based chemotherapy for high-risk stage I tumors 1.
From the Research
Definition and Prevalence of Sex Cord Tumors
- Sex cord-stromal tumors comprise approximately 5% of all testicular tumors, while the remainder are of germ cell origin 2.
- Leydig cell tumors are the most common subtype of testicular sex cord-stromal tumors and account for 1%-2% of all testicular tumors 2.
- Malignant ovarian germ cell tumors and sex cord-stromal tumors each account for less than 5% of all ovarian malignancies 3.
Management and Treatment of Sex Cord Tumors
- Surgical resection of metastasis and/or chemotherapy with bleomycin, etoposide, and cisplatin should be discussed with patients with metastatic Leydig cell tumors, as some have reported complete remission after these interventions 2.
- For patients with ovarian sex cord-stromal tumors, surgery is the main therapeutic modality, while chemotherapy and hormonal therapy may be used in some patients with progressive and recurrent tumors 4.
- The combination of bleomycin, etoposide, and cisplatin has been investigated for the treatment of poor-prognosis sex cord-stromal tumors of the ovary, with an overall response rate of 83% 5.
Prognosis and Outcome of Sex Cord Tumors
- Patients with metastatic Leydig cell tumors have poor prognosis, and standard treatment recommendations do not exist 2.
- The prognosis of ovarian sex cord-stromal tumors may be adversely affected by tumor spillage during surgery or presence of metastases 6.
- For patients with testicular sex cord-stromal tumors, virtually all present as localized nonmetastatic tumors, with excellent prognosis after complete resection 6.
Molecular Changes and Diagnostic Biomarkers
- Understanding molecular changes underlying the development and progression of sex cord-stromal tumors provides valuable information for diagnostic and prognostic biomarkers and potential therapeutic targets for these tumors 4.
- Genetic testing for tumor predisposition syndromes, such as DICER-1, is recommended in selected cases of sex cord-stromal tumors 6.