Management of Granulosa Theca Cell Tumors

Diagnostic Workup

Initial evaluation should include pelvic ultrasound, abdomino-pelvic CT scan, chest X-ray, and in selected cases, PET scan 2
Laboratory tests should include tumor markers such as inhibin B, estradiol, and other relevant tests, and in young patients, complete blood count and liver and renal function tests 2, 3
Histological confirmation by an expert pathologist is essential, with immunohistochemistry panel including inhibin alpha, calretinin, and FOXL2 in morphologically ambiguous cases 2, 4

For young patients desiring fertility preservation, unilateral salpingo-oophorectomy with preservation of the contralateral ovary and uterus is the standard surgical treatment 1, 5, 3
No systematic biopsy of the contralateral ovary is necessary when it appears macroscopically normal 1, 2
Endometrial curettage must be performed to rule out concomitant uterine cancers 1, 3
Retroperitoneal lymph node dissection is not mandatory due to the very low incidence of retroperitoneal metastases in early-stage disease 1, 6, 3

Stage IA adult granulosa cell tumor has excellent prognosis after surgery alone and does not require adjuvant therapy 6, 3, 7
Adjuvant therapy should be considered for patients with high-risk features, such as high mitotic index 5, 6
Platinum-based chemotherapy is the treatment of choice for advanced-stage disease 5, 3, 7
BEP (bleomycin, etoposide, cisplatin) regimen is most commonly used, with three cycles for completely resected disease and four cycles for patients with macroscopic residual disease 3, 7

Debulking surgery is the most effective treatment when feasible 3, 7, 9
For platinum-sensitive relapse, platinum-based combinations should be considered 3, 7
For platinum-resistant disease, options include VAC (vincristine, actinomycin D, cyclophosphamide) and paclitaxel/gemcitabine 3, 7

Follow-up visits should include history, physical examination with pelvic examination, and tumor markers every 3 months for the first 2 years, then every 6 months during years 3-5 5, 3
Long-term follow-up is essential as recurrences can occur up to 20 years after primary diagnosis 8, 9
Pelvic ultrasound and CT scan of abdomen, pelvis, and chest are the most common imaging techniques for surveillance 5, 3
Serum tumor markers including inhibin B, estradiol, and AMH can be useful for monitoring 5, 8, 9