Progress in the management of ovarian granulosa cell tumor: A review

Acta Obstet Gynecol Scand. 2021 Oct;100(10):1771-1778. doi: 10.1111/aogs.14189. Epub 2021 Jul 16.


Ovarian granulosa cell tumor (GCT) is a rare, low-grade malignant tumor that accounts for 70% of the sex cord-stromal tumors. It has two histopathologic types with different clinical and biologic features: adult GCT and juvenile GCT. Most women diagnosed with the adult GCT have a favorable prognosis, with a 5-year survival rate of 97%-98%, but adult GCT has a feature of late relapse; the recurrence time could be more than 20 years after diagnosis. Juvenile GCT has a survival rate of 97% in stage I and a 5-year survival rate of 0%-22% in advanced stage with earlier recurrence than adult GCT. Consequently, the scenario emphasizes the need for early diagnosis, standardized treatment protocols, and long-term follow up. However, there is a lack of consensus regarding accurate diagnosis of GCT and adjuvant treatment. Furthermore, GCT tends to occur in young women, which emphasizes the viability of fertility-sparing surgery. The current review performed a systematic literature review of 60 articles to summarize the latest advances in GCT, with an emphasis on the molecular pathogenesis and survival after fertility-sparing surgery. We found that young women with fertility-sparing surgery had a desirable reproductive and survival outcome compared with those undergoing radical surgery.

Keywords: fertility-sparing surgery; forkhead box L2 mutation; granulosa cell tumor of the ovary; molecular diagnosis; sex cord-stromal tumor.

Publication types

  • Systematic Review

MeSH terms

  • Female
  • Fertility Preservation*
  • Granulosa Cell Tumor / mortality
  • Granulosa Cell Tumor / surgery*
  • Humans
  • Neoplasm Recurrence, Local / mortality
  • Neoplasm Recurrence, Local / surgery*
  • Ovarian Neoplasms / mortality
  • Ovarian Neoplasms / surgery*
  • Survival Analysis

Supplementary concepts

  • Granulosa cell tumor of the ovary