NCI Definition: A granulosa-stromal cell tumor that arises from the ovary. It is characterized by the presence of granulosa cells that comprise at least ten percent of the cellular population. The granulosa cells are often found in a background that contains theca and fibrous cells. There are two major subtypes recognized, adult and juvenile granulosa cell tumor. Clinically, patients may present with an abdominal mass. Symptoms depend on the patient's age. The most important indicator of prognosis is tumor stage. Age over forty years at the time of the initial diagnosis, large tumor size, bilaterality, cellular atypia, and increased mitotic activity are factors indicating a potentially aggressive clinical course and relative poor prognosis. [1]

Ovarian granulosa cell tumors most frequently harbor alterations in KMT2D [2].

Most Commonly Altered Genes in Ovarian Granulosa Cell Tumor

KMT2D Mutation are the most common alterations in ovarian granulosa cell tumor [2].

Top Alterations in Ovarian Granulosa Cell Tumor

Disease Details

Ovarian Granulosa Cell Neoplasm, Granulosa Cell Tumor of the Ovary, Ovary granulosa cell tumor, Granulosa Cell Neoplasm of Ovary, Granulosa Cell Neoplasm of the Ovary
Granulosa Cell Tumor
OncoTree Name
Granulosa Cell 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 6. 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.