Ovarian sex cord-stromal tumors: an update on clinical features, molecular changes, and management

Int J Gynecol Cancer. 2021 Feb;31(2):161-168. doi: 10.1136/ijgc-2020-002018. Epub 2021 Jan 7.


Sex cord stromal-tumors are rare tumors of the ovary that include numerous tumor subtypes of variable histological features and biological behavior. Surgery is the main therapeutic modality for the management of these tumors, while chemotherapy and hormonal therapy may be used in some patients with progressive and recurrent tumors. Several studies investigated molecular changes in the different tumor types. Understanding molecular changes underlying the development and progression of sex cord-stromal tumors provides valuable information for diagnostic and prognostic biomarkers and potential therapeutic targets for these tumors. In this review, we provide an update on the clinical presentation, molecular changes, and management of sex cord-stromal tumors.

Keywords: ovarian cancer; sex cord-gonadal stromal tumors.

Publication types

  • Review

MeSH terms

  • Biomarkers, Tumor
  • Diagnosis, Differential
  • Female
  • Humans
  • Neoplasm Recurrence, Local / diagnosis
  • Neoplasm Recurrence, Local / pathology
  • Neoplasm Recurrence, Local / therapy
  • Ovarian Neoplasms / diagnosis
  • Ovarian Neoplasms / pathology*
  • Ovarian Neoplasms / therapy
  • Sex Cord-Gonadal Stromal Tumors / diagnosis
  • Sex Cord-Gonadal Stromal Tumors / pathology*
  • Sex Cord-Gonadal Stromal Tumors / therapy


  • Biomarkers, Tumor