Endometrioid stromal sarcoma

Summary

Endometrioid stromal sarcoma (MONDO:0006745) is a cancer and 8 clinical trials. Top therapeutic interventions include elacestrant, fulvestrant, and saracatinib. A subtype of endometrial stromal tumor — broader associated-gene and molecular evidence is on the parent page (see Disease family below).

At a glance

• Classification: Cancer
• Clinical trials: 8

Clinical features

No curated clinical features (Orphanet) for this disease.

Identifiers

Disease identifiers

Field	Value
Canonical name	endometrioid stromal sarcoma
Mondo ID	MONDO:0006745
EFO	EFO:1000919
MeSH	D018203
Orphanet	213711
DOID	DOID:4226
NCIT	C8973
SNOMED CT	699356008
UMLS	C0206630
MedGen	60196
GARD	0006339
MedDRA	10048397, 10057649
NORD	1086
Is cancer (heuristic)	yes

Also known as: endometrial stromal sarcoma · endometrial stromal sarcomas · endometrioid stromal sarcoma · ESS · ess · sarcomas, endometrial stromal · stromal sarcoma of the corpus uteri · stromal sarcoma, endometrial · stromal sarcoma, endometrial, malignant · stromal sarcomas, endometrial

Data availability: 6 cell lines.

Disease family

This is a subtype of endometrial stromal tumor. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.

Classification path: disease › human disease › disease by body system or component › reproductive system disorder › female reproductive system disorder › uterine disorder › endometrial disorder › endometrium neoplasm › endometrial stromal tumor › endometrioid stromal sarcoma

Subtypes (3): uterine corpus endometrial stromal sarcoma, endometrioid stromal sarcoma of the cervix, endometrioid stromal sarcoma of the vagina

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

Drugs indicated for this disease

No approved or late-stage (phase ≥3) drug is indicated for this disease; the following are in earlier-phase trials only.

Earlier-phase candidates (phase 2, investigational — efficacy not yet established): Fulvestrant.

Clinical trials & evidence

Clinical trials

Clinical trials: 8.

Phase distribution (across all retrieved trials)

Phase	Trials
PHASE2	6
PHASE1	1
Not specified	1

Top trials by phase / activity

NCT	Phase	Status	Title
NCT06277154	PHASE2	RECRUITING	MASCT-I Combined With Doxorubicin and Ifosfamide for First-line Treatment of Advanced Soft Tissue Sarcoma
NCT07467772	PHASE2	RECRUITING	Ph 2 Elacestrant in ER Positive Uterine Sarcomas
NCT00659360	PHASE2	COMPLETED	AZD0530 in Treating Patients With Recurrent Locally Advanced or Metastatic Soft Tissue Sarcoma
NCT01210222	PHASE2	COMPLETED	Trebananib in Treating Patients With Persistent or Recurrent Endometrial Cancer
NCT02150213	PHASE2	COMPLETED	Medical Safety Follow-up Study for Patients Who Received More Than 28 Days of Total Exposure to BGG492
NCT03926936	PHASE2	COMPLETED	FUlvestrant in Gynecological Cancers That Are Potentially Hormone Sensitive: the FUCHSia Study
NCT04811196	PHASE1	COMPLETED	A Study of Different Dosing Schedules of Selinexor in Sarcoma Patients
NCT00897442	Not specified	COMPLETED	Collecting Tumor Samples From Patients With Gynecological Tumors

Drugs tested across these trials (top 30)

Molecule	Max phase	Trials referencing
ELACESTRANT	4	1
FULVESTRANT	4	1
SARACATINIB	3	1
TREBANANIB	3	1
SELURAMPANEL	2	1
CHEMBL4438584	0	1