From the Guidelines
The primary treatment for granulosa cell tumors is surgical resection, typically involving total hysterectomy with bilateral salpingo-oophorectomy for adult patients who have completed childbearing, and debulking surgery remains the most effective treatment for metastatic or recurrent granulosa cell tumors 1. For younger patients desiring fertility preservation, unilateral salpingo-oophorectomy with comprehensive surgical staging may be considered for early-stage disease. Surgical staging should include:
- Peritoneal washings
- Omental sampling
- Assessment of pelvic and para-aortic lymph nodes Adjuvant chemotherapy is recommended for advanced or recurrent disease, with the BEP regimen (bleomycin, etoposide, and cisplatin) being the most commonly used protocol, typically administered for 3-4 cycles, or carboplatin/paclitaxel for six cycles 1. Hormonal therapy with agents such as leuprolide acetate, megestrol acetate, or aromatase inhibitors like letrozole may be effective for recurrent disease due to the hormone-responsive nature of these tumors, with a complete response rate of 25.8% and a partial response rate of 45.2% 1. Long-term surveillance is essential as granulosa cell tumors can recur many years after initial treatment, requiring tumor marker monitoring (inhibin B and AMH) and regular imaging studies for at least 10 years. The effectiveness of these treatments is based on the tumor's unique characteristics, including its typically indolent growth pattern, hormone production capabilities, and tendency for late recurrence. Alternative chemotherapy options include PVB, EP, CAP, VAC, and weekly paclitaxel for relapsed patients after platinum-based chemotherapy 1. Anti-angiogenic agents such as bevacizumab have also been investigated in patients with recurrent granulosa cell tumors, with a response rate of 17% and stable disease in 77% of patients 1.
From the Research
Treatment Options for Granulosa Cell Tumor (GCT)
The treatment for Granulosa Cell Tumor (GCT) includes:
- Surgery, which is the standard treatment, including fertility-sparing procedures for young women 2, 3
- Adjuvant treatments, such as chemotherapy and radiotherapy, which are used to treat advanced and/or recurrent disease 2, 3
- Chemotherapy regimens, such as BEP (bleomycin, etoposide, and cisplatin), which are commonly used to treat GCT 2, 3
- Anti-hormonal therapy, such as aromatase inhibitors, which may be a viable alternative to traditional treatment modalities 4, 5
- Hyperthermic intraperitoneal chemotherapy (HIPEC), which has been proposed as a novel therapeutic option for recurrent GCT 6
Response to Systemic Therapies
The response to systemic therapies in GCT is limited, with:
- Objective response rate (ORR) of 30% and disease control rate (DCR) of 58% for chemotherapy regimens 5
- ORR of 11% and DCR of 66% for anti-hormonal therapy regimens 5
- Limited evidence on the effectiveness of systemic therapy in GCT, highlighting the need for new approaches and standardized treatment protocols 5
Recurrent GCT Treatment
The treatment of recurrent GCT is not standardized, but options include: