NCI Definition: A benign or malignant neoplasm that arises from the ovary and is composed of granulosa cells, Sertoli cells, Leydig cells, theca cells, and fibroblasts. Representative examples include thecoma, fibroma, Sertoli cell tumor, and granulosa cell tumor. [1]

Ovarian sex cord stromal tumors most frequently harbor alterations in FOXL2, DICER1, KMT2D, PRKDC, and ATM [2].

Most Commonly Altered Genes in Ovarian Sex Cord Stromal Tumor

FOXL2 Mutation, FOXL2 C134W, DICER1 Mutation, KMT2fs, and KMT2D Mutation are the most common alterations in ovarian sex cord stromal tumor [2].

Top Alterations in Ovarian Sex Cord Stromal Tumor

Disease Details

Ovarian Sex Cord-Stromal Neoplasm, Ovarian Sex Cord-Stromal Tumor, Sex Cord-Stromal Tumor of the Ovary, Sex Cord-Stromal Neoplasm of the Ovary, Sex Cord-Stromal Neoplasm of Ovary, Sex Cord-Stromal Tumor of Ovary
Sex Cord-Stromal Tumor
Ovarian Steroid Cell Tumor, Ovarian Sex Cord Tumor with Annular Tubules, Ovarian Granulosa-Stromal Cell Tumor, and Ovarian Sertoli-Stromal Tumor
OncoTree Name
Sex Cord Stromal Tumor
OncoTree Code


1. National Cancer Institute. NCI Thesaurus Version 18.11d. https://ncit.nci.nih.gov/ncitbrowser/ [2018-08-28]. [2018-09-21].

2. The AACR Project GENIE Consortium. AACR Project GENIE: powering precision medicine through an international consortium. Cancer Discovery. 2017;7(8):818-831. Dataset Version 8. This dataset does not represent the totality of the genetic landscape; see paper for more information.

3. All assertions and clinical trial landscape data are curated from primary sources. You can read more about the curation process here.