Maligant granulosa cell tumor of ovary

Summary

Maligant granulosa cell tumor of ovary (MONDO:0020541) is a cancer and 2 clinical trials. Top therapeutic interventions include darolutamide, exemestane, and luspatercept. A subtype of malignant sex cord stromal tumor of ovary — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Classification: Cancer
• Prevalence: 1-9 / 1 000 000 (Europe) [Orphanet-validated]
• Clinical trials: 2

Clinical features

Epidemiology

Prevalence records

1 prevalence record(s), Orphanet:

Type	Class	Value	Geography	Validation
Annual incidence	1-9 / 1 000 000	0.12	Europe	Validated

Identifiers

Disease identifiers

Field	Value
Canonical name	maligant granulosa cell tumor of ovary
Mondo ID	MONDO:0020541
Orphanet	99915
ICD-11	148207042
NCIT	C8403
SNOMED CT	254861002
UMLS	C0346175
MedGen	138043
GARD	0019707
Is cancer (heuristic)	yes

Also known as: adult granulosa cell tumor of the ovary · adult granulosa cell tumour of the ovary · adult ovarian granulosa cell tumor · adult ovarian granulosa cell tumour · cancer of granulosa cell · GCT of the ovary · granulosa cell cancer · granulosa cell malignant tumor · granulosa cell malignant tumour · granulosa theca cell tumor · granulosa theca cell tumor of the ovary · granulosa theca cell tumour · granulosa theca cell tumour of the ovary · GTCT · Maligant granulosa cell tumour of the ovary · malignant granulosa cell neoplasm · malignant granulosa cell neoplasm of ovary · malignant granulosa cell neoplasm of the ovary · malignant granulosa cell tumor of ovary · malignant granulosa cell tumor of the ovary (+7 more)

Disease family

Classification path: disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › cancer › reproductive system cancer › female reproductive organ cancer › ovarian cancer › malignant non-epithelial tumor of ovary › malignant sex cord stromal tumor of ovary › maligant granulosa cell tumor of ovary

Related subtypes (3): ovarian gonadoblastoma, malignant Sertoli-Leydig cell tumor of ovary, theca steroid-producing cell malignant tumor of ovary, not further specified

Genetics & variants

GWAS landscape

No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.

Variant details and genetic-evidence tiers

No tiered GWAS variants or ClinVar records for this disease.

Genes & proteins

No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).

Function

No pathway enrichment — requires an associated-gene cohort.

Therapeutics

No druggable-target or therapeutic data for this disease’s cohort.

Clinical trials & evidence

Clinical trials

Clinical trials: 2.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	1
Not specified	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT06169124	PHASE2	ACTIVE_NOT_RECRUITING	Study to Test the Drug Darolutamide Along With the Drugs Leuprolide Acetate and Exemestane in Patients With Recurrent Ovarian Granulosa Cell Tumors
NCT06254781	Not specified	COMPLETED	Luspatercept in Metastatic AGCT of the Ovary

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
DAROLUTAMIDE	4	1
EXEMESTANE	4	1
LUSPATERCEPT	4	1
CHEMBL4457117	0	1
CHEMBL5206636	0	1