Management of interstitial tumors of the ovarian sex cord

Interstitial cell tumors of the ovarian sex cord can be divided into: granulosa cell tumors, follicular membrane cell tumors, supportive cell tumors, mesenchymal cell tumors, granulosa cell-follicular membrane cell tumors, supportive-mesenchymal cell tumors, and amphiblastomas. Granulosa cell tumors account for 80% of interstitial tumors of the sex cord and 3-5% of all ovarian tumors. Granulosa cell tumors can be divided into adult granulosa cell tumors and juvenile granulosa cell tumors. Granulosa cell tumors are slow to develop and are mostly at an early stage of diagnosis and have a better prognosis. Because granulosa cells can produce estrogen, patients with juvenile granulosa cell tumors often present with precocious puberty, while adult granulosa cell tumors may present with postmenopausal vaginal bleeding, endometrial hyperplasia, and endometrial cancer, so diagnostic curettage should be performed when these patients are suspected. Support – Mesenchymal cell tumors can secrete androgens and patients may exhibit masculine features. Treatment principles: Among them, except for granulosa cell tumor, which is a low-grade malignant tumor, and moderately differentiated and poorly differentiated supporting-mesenchymal cell tumors, which are logarithmically malignant, most of the other types of ovarian sex cord mesenchymal tumors are absolutely benign and should be treated according to the principles of benign ovarian tumors. The period of diagnosis, size of the tumor, and histologic type are key prognostic factors. The main treatment modalities are surgery and chemotherapy. Patients with sex cord-mesenchymal tumor who wish to preserve their reproductive function and are confined to one ovary are eligible for full-scale staging surgery to preserve their reproductive function. All other patients are recommended to undergo full-stage surgery without lymph node dissection. Patients with preserved fertility may be monitored postoperatively with ultrasound. Consider radical surgery after completion of childbearing (level 2B evidence). ①Stage I low-risk patients may be observed postoperatively only. ②Stage I high-risk patients (tumor rupture, stage IC, poorly differentiated, tumor diameter more than 10-15 cm) or intermediate-risk patients (heterologous component) may be selected (level 2B evidence): observation, radiotherapy, or platinum-based chemotherapy. If inhibin levels are elevated prior to treatment, inhibin levels should be monitored for follow-up (level 2B evidence). 2015 FIGO report states that there is no evidence to support that postoperative adjuvant chemotherapy and radiotherapy improve the prognosis of stage I patients, and the value of postoperative adjuvant chemotherapy for stage I patients with high-risk factors is uncertain. (iii) Stage II-IV patients have the option (all level 2B evidence) of radiation therapy to limited lesions or platinum-based chemotherapy (BEP regimen or paclitaxel + carboplatin regimen preferred). Patients with stage II-IV disease who experience clinical relapse after completion of treatment may choose to participate in a clinical trial or follow a relapse regimen, or may be considered for re-tumor cytoreductive surgery. For advanced or recurrent granulosa cell tumors, platinum-based chemotherapy can achieve 63-80% overall response rates. Bevacizumab and leuprolide may be used to treat recurrent granulosa cell tumors. 2. Follow-up Patients with granulosa cell tumors can experience late recurrence (e.g., recurrence after 30 years), and extended follow-up of these patients is recommended. If suppressor B and Anti Miillerian Hormone (AMH) are elevated at the time of diagnosis, suppressor B and AMH can be used as indicators of early detection of residual or recurrent lesions during follow-up. Blood inhibin can be used as a valid tumor marker in menopausal women (level C evidence).