Systemic Therapy for Non-Serous Ovarian Carcinoma

Yanin Chávarri-Guerra; Eduardo González-Ochoa; Héctor De-la-Mora-Molina; Enrique Soto-Perez-de-Celis


Chin Clin Oncol. 2020;9(4):52 

In This Article

Malignant Sex Cord-stromal Tumors

Sex cord-stromal tumors are infrequent, representing only 7% of all ovarian tumors. These tumors arise from the primitive sex cords or stromal cells, with the most common being those derived from the granulosa, followed by Sertoli-Leydig cell tumors. Most present as low grade and are of good prognosis, presenting more frequently in younger patients, with the exception being the adult granulosa cell tumor, which presents later in life (up to age 55 years).[79] Sex cord-stromal tumors often have hormone-associated symptoms (including virilization and hypoestrogenism) due to their production of androgens, estrogens and corticoids. Histologic classification includes pure sex cord tumors, pure stromal tumors, and mixed tumors.[80] Mutations in DICER1, STK11 and FOXL2 can be found in some of these tumors.

The primary treatment is surgery. Fertility-sparing surgery is recommended for patients desiring to preserve their fertility and with early stage tumors (stage IA or IC). Complete staging (sampling of peritoneal fluid, examination of the contralateral ovary, biopsies of the peritoneum and any suspicious lesions, omental biopsy, and palpation of lymph nodes) is recommended for all other patients.[29,79] Adjuvant treatment includes radiotherapy for limited disease and platinum-based chemotherapy for patients with stage II to IV disease. Due to their rarity, few studies have looked at the efficacy of adjuvant therapy[81] (Table 3). Currently, the most commonly utilized chemotherapy regimen is BEP for four to six cycles,[82,83] although the combination of paclitaxel plus carboplatin has also shown to be an active regimen.[84,85]

Relapse is uncommon, however for those that have a recurrence systemic therapy options include single agent taxanes or taxanes in combination with a platinum compound or ifosfamide. Endocrine therapy, including aromatase inhibitors, tamoxifen, and leuprolide, has also shown efficacy.[86] Bevacizumab alone or in combination is another therapeutic option for recurrent disease.[87,88]