ESS
MCID: END031
MIFTS: 46

Endometrial Stromal Sarcoma (ESS)

Categories: Cancer diseases, Endocrine diseases, Rare diseases, Reproductive diseases

Aliases & Classifications for Endometrial Stromal Sarcoma

MalaCards integrated aliases for Endometrial Stromal Sarcoma:

Name: Endometrial Stromal Sarcoma 12 74 52 58 15 17 71
Ess 12 52
Endometrial Stromal Sarcoma, High Grade 12
Undifferentiated Endometrial Sarcoma 12
Stromal Sarcoma of the Corpus Uteri 58
Undifferentiated Stromal Sarcoma 71
Sarcoma, Endometrial Stromal 43
Sarcoma Endometrial Stromal 54

Classifications:

Orphanet: 58  
Rare gynaecological and obstetric diseases


External Ids:

Disease Ontology 12 DOID:4226
MeSH 43 D018203
NCIt 49 C8973
SNOMED-CT 67 699356008
MESH via Orphanet 44 D018203
ICD10 via Orphanet 33 C54.1
UMLS via Orphanet 72 C0206630
Orphanet 58 ORPHA213711
UMLS 71 C0206630 C1710535

Summaries for Endometrial Stromal Sarcoma

NIH Rare Diseases : 52 Endometrial stromal sarcoma is a rare form of cancer that occurs due to abnormal and uncontrolled cell growth in the uterus. Endometrial stromal sarcoma, specifically, develops in the supporting connective tissue (stroma) of the uterus. Signs and symptoms of the condition include abnormal uterine bleeding (i.e. bleeding that is not part of menstrual periods or bleeding after menopause); abdominal pain and/or distension; and frequent urination. The exact underlying cause of endometrial stromal sarcoma is currently unknown. Most cases occur sporadically in people with no family history of the condition. Treatment varies based on the severity of the condition but may include surgery, radiation therapy , chemotherapy , and/or hormone therapy .

MalaCards based summary : Endometrial Stromal Sarcoma, also known as ess, is related to vaginal endometrial stromal sarcoma and endometrial stromal nodule. An important gene associated with Endometrial Stromal Sarcoma is SUZ12 (SUZ12 Polycomb Repressive Complex 2 Subunit), and among its related pathways/superpathways are Striated Muscle Contraction and Signaling events mediated by HDAC Class II. The drugs Doxorubicin and Lenograstim have been mentioned in the context of this disorder. Affiliated tissues include uterus, smooth muscle and lung, and related phenotypes are cardiovascular system and cellular

Disease Ontology : 12 An endometrial stromal tumor that has material basis in connective tissue.

Wikipedia : 74 Endometrial stromal sarcoma is a malignant subtype of endometrial stromal tumor arising from the stroma... more...

Related Diseases for Endometrial Stromal Sarcoma

Diseases related to Endometrial Stromal Sarcoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 345)
# Related Disease Score Top Affiliating Genes
1 vaginal endometrial stromal sarcoma 35.2 YWHAE VIM SUZ12 PHF1 PGR MME
2 endometrial stromal nodule 33.8 ZC3H7B YWHAE SUZ12 PHF1 NUTM2A MME
3 sarcoma 33.2 VIM SUZ12 KIT DES ACTC1
4 spindle cell sarcoma 33.2 VIM DES ACTC1
5 leiomyosarcoma 32.4 ZC3H7B VIM PGR KIT ESR1 DES
6 leiomyoma 32.4 VIM PGR MME KIT ESR1 DES
7 rare tumor 32.3 KIT DES ACTC1
8 adenomyosis 32.1 PGR MME ESR1 CYP19A1
9 mesenchymal cell neoplasm 32.1 ZC3H7B MME KIT BCOR
10 endometrial stromal tumor 32.1 ZC3H7B YWHAE SUZ12 PHF1 PGR NUTM2B
11 myoma 32.1 PGR MME KIT ESR1 CYP19A1
12 cellular leiomyoma 32.0 SUZ12 MME JAZF1 DES
13 carcinosarcoma 32.0 VIM PGR MME KIT DES ACTC1
14 leiomyomatosis 32.0 PGR KIT ESR1
15 intravenous leiomyomatosis 32.0 PGR MME ESR1 DES
16 smooth muscle tumor 31.8 VIM PGR MME KIT ESR1 DES
17 myxoid leiomyosarcoma 31.8 ZC3H7B YWHAE PGR MEAF6 EZHIP BCOR
18 neurilemmoma 31.7 VIM KIT DES ACTC1
19 adenosarcoma 31.7 YWHAE VIM PHF1 PGR MME KIT
20 hemangiopericytoma, malignant 31.7 VIM KIT DES ACTC1
21 sarcoma, synovial 31.6 VIM KIT DES BCOR
22 endometriosis of ovary 31.6 PGR ESR1 CYP19A1
23 pneumothorax 31.6 VIM PGR ACTC1
24 endometrial adenocarcinoma 31.6 VIM PGR MALAT1 ESR1
25 leiomyoma, uterine 31.6 PGR ESR1 CYP19A1
26 uterine carcinosarcoma 31.6 VIM PGR MME KIT ESR1 DES
27 cervical polyp 31.5 PGR MME DES
28 lymphangioleiomyomatosis 31.5 PGR ESR1 DES
29 adenomyoma 31.5 PGR MME ESR1
30 embryonal sarcoma 31.5 VIM MALAT1 KIT DES
31 gastrointestinal stromal tumor 31.4 VIM KIT DES ACTC1
32 endometrial hyperplasia 31.3 PGR ESR1 CYP19A1
33 benign metastasizing leiomyoma 31.3 VIM PGR DES CYP19A1 ACTC1
34 fibroma 31.2 VIM MALAT1 DES ACTC1
35 perivascular epithelioid cell tumor 31.2 PGR MME KIT DES ACTC1
36 adenofibroma 31.2 VIM MME ACTC1
37 glomus tumor 31.2 VIM DES ACTC1
38 inflammatory myofibroblastic tumor 31.2 VIM KIT DES ACTC1
39 ewing sarcoma 31.0 VIM KIT DES BCOR
40 cystadenocarcinoma 31.0 VIM PGR ACTC1
41 uterine corpus endometrial stromal sarcoma 12.6
42 cervical endometrial stromal sarcoma 12.6
43 empty sella syndrome 12.2
44 euthyroid sick syndrome 11.3
45 endometriosis 10.9
46 lymphangiomatosis 10.9 VIM PGR DES
47 uterine corpus adenosarcoma 10.9 PGR MME ESR1
48 congenital epulis 10.9 VIM PGR DES
49 epulis 10.9 VIM PGR DES
50 mammographic density 10.9 PGR ESR1 CYP19A1

Graphical network of the top 20 diseases related to Endometrial Stromal Sarcoma:



Diseases related to Endometrial Stromal Sarcoma

Symptoms & Phenotypes for Endometrial Stromal Sarcoma

MGI Mouse Phenotypes related to Endometrial Stromal Sarcoma:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.85 ACTC1 BCOR CYP19A1 DES ESR1 KIT
2 cellular MP:0005384 9.7 ACTC1 BCOR CYP19A1 DES ESR1 KIT
3 muscle MP:0005369 9.23 ACTC1 CYP19A1 DES ESR1 KIT PGR

Drugs & Therapeutics for Endometrial Stromal Sarcoma

Drugs for Endometrial Stromal Sarcoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 61)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Doxorubicin Approved, Investigational Phase 3 23214-92-8 31703
2
Lenograstim Approved, Investigational Phase 3 135968-09-1
3
Ifosfamide Approved Phase 3 3778-73-2 3690
4
Histamine Approved, Investigational Phase 3 51-45-6 774
5
Cyproheptadine Approved Phase 3 129-03-3 2913
6 Anti-Bacterial Agents Phase 3
7
Liposomal doxorubicin Phase 3 31703
8 Antibiotics, Antitubercular Phase 3
9 Alkylating Agents Phase 3
10
Isophosphamide mustard Phase 3 0
11 Gastrointestinal Agents Phase 3
12 Neurotransmitter Agents Phase 3
13 Histamine Antagonists Phase 3
14 Anti-Allergic Agents Phase 3
15
Histamine Phosphate Phase 3 51-74-1 65513
16 Histamine H1 Antagonists Phase 3
17 Dermatologic Agents Phase 3
18
Serotonin Investigational, Nutraceutical Phase 3 50-67-9 5202
19
Temozolomide Approved, Investigational Phase 2 85622-93-1 5394
20
Hydrocortisone Approved, Vet_approved Phase 2 50-23-7 5754
21
Hydrocortisone acetate Approved, Vet_approved Phase 2 50-03-3
22
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
23
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
24
Trabectedin Approved, Investigational Phase 2 114899-77-3 108150
25
Paclitaxel Approved, Vet_approved Phase 2 33069-62-4 36314
26
Sunitinib Approved, Investigational Phase 2 557795-19-4, 341031-54-7 5329102
27
Fulvestrant Approved, Investigational Phase 2 129453-61-8 104741 17756771
28
Letrozole Approved, Investigational Phase 2 112809-51-5 3902
29
Exemestane Approved, Investigational Phase 2 107868-30-4 60198
30
Anastrozole Approved, Investigational Phase 2 120511-73-1 2187
31
nivolumab Approved Phase 2 946414-94-4
32
Saracatinib Investigational Phase 2 379231-04-6
33
Trebananib Investigational Phase 2 894356-79-7
34 Angiogenesis Inhibitors Phase 2
35 Hydrocortisone 17-butyrate 21-propionate Phase 2
36 Antiemetics Phase 2
37 Hydrocortisone hemisuccinate Phase 2
38 glucocorticoids Phase 2
39 Anti-Inflammatory Agents Phase 2
40 Immunologic Factors Phase 2
41 Immunosuppressive Agents Phase 2
42 Anti-Infective Agents Phase 2
43 Albumin-Bound Paclitaxel Phase 2
44 Tubulin Modulators Phase 2
45 Antimitotic Agents Phase 2
46 Protein Kinase Inhibitors Phase 1, Phase 2
47 Imatinib Mesylate Phase 1, Phase 2 220127-57-1 123596
48 Antineoplastic Agents, Hormonal Phase 2
49 Estrogen Antagonists Phase 2
50 Estrogen Receptor Antagonists Phase 2

Interventional clinical trials:

(show all 23)
# Name Status NCT ID Phase Drugs
1 Phase III Randomized Study of Adjuvant Pelvic Radiotherapy Versus Observation Alone in Patients With Completely Resected, Stage I or II, High-Grade Uterine Sarcoma Completed NCT00002459 Phase 3
2 METASTASECTOMY AND CHEMOTHERAPY FOR LUNG METASTASES FROM SOFT TISSUE SARCOMA: A RANDOMIZED PHASE III STUDY (AN INTERGROUP STUDY WITH THE SCANDINAVIAN SARCOMA GROUP) Completed NCT00002764 Phase 3 doxorubicin hydrochloride;ifosfamide
3 RANDOMISED TRIAL OF ADJUVANT CHEMOTHERAPY WITH HIGH-DOSE DOXORUBICIN, IFOSFAMIDE AND LENOGRASTIM IN HIGH GRADE SOFT TISSUE SARCOMA Completed NCT00002641 Phase 3 doxorubicin hydrochloride;ifosfamide;isolated perfusion
4 Prevention of Cancer/Treatment-Related Weight Loss in Children at High Nutritional Risk Terminated NCT01132547 Phase 3 cyproheptadine hydrochloride
5 Phase II Evaluation of Temodal (Temozolomide, Schering) in Previously Treated Advanced Sarcomas Unknown status NCT00003718 Phase 2 temozolomide
6 A Phase II Study Of Perifosine (D-21266) In Patients With Previously Untreated Metastatic Or Locally Advanced Soft Tissue Sarcoma Completed NCT00053794 Phase 2 perifosine
7 Multicenter Parallel Phase II Trial of BI 2536 Administered as One Hour IV Infusion Every 3 Weeks in Defined Cohorts of Patients With Various Solid Tumors. A New Drug Screening Program of the EORTC Network of Core Institutions (NOCI) Completed NCT00526149 Phase 2 BI 2536
8 A Phase 2 Study of AZD0530 in Recurrent or Metastatic Soft Tissue Sarcoma Completed NCT00659360 Phase 2 saracatinib
9 A Phase II Trial of AMG 386, a Selective Angiopoietin 1/2 Neutralizing Peptibody, in Patients With Persistent/Recurrent Carcinoma of the Endometrium Completed NCT01210222 Phase 2
10 A Multicenter Medical Safety Follow-up Study for Patients With Partial Onset Seizures Who Received More Than 28 Days of Total Exposure to BGG492 in Studies CBGG492A2207 and/or CBGG492A2212 Completed NCT02150213 Phase 2 BGG492
11 Phase II Study of Mifepristone (RU-486) in the Treatment of PR Positive Advanced/Recurrent Endometrioid Adenocarcinoma and Low Grade Endometrial Stromal Sarcoma (LGESS) Completed NCT00505739 Phase 2 Mifepristone
12 Phase II Study of Thalidomide (NSC #66847) in Patients With Sarcomas of Gynecologic Origin Completed NCT00006005 Phase 2 thalidomide
13 Evaluation of Doxil in the Treatment of Recurrent or Advanced Leiomyosarcoma of the Uterus Completed NCT00005643 Phase 2 pegylated liposomal doxorubicin hydrochloride
14 Phase II Study on ET-743 in Advanced Soft Tissue Sarcomas of the Adult Completed NCT00003939 Phase 2 trabectedin
15 Phase II Study of Doxil and Paclitaxel in Patients With Endometrial, Tubal, and Sarcomas of Gynecologic Origin Completed NCT00003334 Phase 2 paclitaxel;pegylated liposomal doxorubicin hydrochloride
16 Dose Finding and Phase II Study of STI 571 in Advanced Soft Tissue Sarcoma Completed NCT00006357 Phase 1, Phase 2 imatinib mesylate
17 A Multicenter Phase II Study of Continuous Dosing of Sunitinib (Sutent®, SU11248) in Non-GIST Sarcomas Completed NCT00474994 Phase 2 sunitinib malate
18 An Open-label, Single Arm, Prospective, Multi-center, Tandem Two Stage Designed, Phase II Study to Evaluate the Efficacy of Fulvestrant in Women With Recurrent/Metastatic Estrogen Receptor Positive Gynecological Malignancies Recruiting NCT03926936 Phase 2 Fulvestrant
19 Randomized Comparative Prospective Multicentre Phase II Trial Evaluating Clinical Impact of Interruption VS Maintenance of AI in Patients With Locally Advanced/ Metastatic LGESS Recruiting NCT03624244 Phase 2 Aromatase Inhibitors
20 Phase II Trial of Single-Agent Nivolumab in Patients With Microsatellite Unstable/Mismatch Repair Deficient/Hypermutated Uterine Cancer Recruiting NCT03241745 Phase 2 Nivolumab
21 Acquisition of Human Gynecologic Specimens to be Used in Studying the Causes, Diagnosis, Prevention and Treatment of Cancer Completed NCT00897442
22 MYometrial Lesion UltrasouNd And mRi Recruiting NCT04123158
23 INST 0817: Compassionate Use of Mifepristone Terminated NCT00832871 Mifepristone

Search NIH Clinical Center for Endometrial Stromal Sarcoma

Cochrane evidence based reviews: sarcoma, endometrial stromal

Genetic Tests for Endometrial Stromal Sarcoma

Anatomical Context for Endometrial Stromal Sarcoma

MalaCards organs/tissues related to Endometrial Stromal Sarcoma:

40
Uterus, Smooth Muscle, Lung, Ovary, Cervix, Colon, Breast

Publications for Endometrial Stromal Sarcoma

Articles related to Endometrial Stromal Sarcoma:

(show top 50) (show all 1018)
# Title Authors PMID Year
1
[Immunohistochemical study of endometrial stromal sarcoma and smooth-muscle tumors of the uterus]. 54 61
18565690 2008
2
Aromatase inhibitors in gynecologic cancers. 54 61
17826626 2007
3
Hormonal therapy of endometrial stromal sarcoma. 54 61
17545798 2007
4
Pulmonary metastasis of endometrial stromal sarcoma. 61 54
17317938 2006
5
Hormonal treatment with aromatase inhibitors for patients with endometrial stromal sarcoma. 61 54
15963815 2005
6
Utility of MIB-1 and estrogen and progesterone receptor in distinguishing between endometrial stromal sarcomas and endometrial stromal nodules, highly cellular leiomyomas. 61 54
15823122 2005
7
Distinction of endometrial stromal sarcomas from 'hemangiopericytomatous' tumors using a panel of immunohistochemical stains. 61 54
15309019 2005
8
Treatment of endometrial stromal sarcoma with a gonadotropin-releasing hormone analogue. 61 54
15516445 2004
9
Recurrence of prolactin-producing endometrial stromal sarcoma with sex-cord stromal component treated with progestin and aromatase inhibitor. 61 54
15297205 2004
10
Expression of progesterone receptor A and B isoforms in low-grade endometrial stromal sarcoma. 61 54
15084842 2004
11
Low-grade endometrial stromal sarcoma: hormonal aspects. 54 61
12821359 2003
12
Expression of CD10 in malignant müllerian mixed tumors and adenosarcomas: an immunohistochemical study. 54 61
12218209 2002
13
Cytologic diagnosis of low grade endometrial stromal sarcoma by staining for estrogen and progesterone receptors. 54 61
12146057 2002
14
Immunohistochemical staining for KIT (CD117) in soft tissue sarcomas is very limited in distribution. 61 54
11865845 2002
15
Endometrial stromal sarcoma: objective response to letrozole. 61 54
11531300 2001
16
Utility of CD10 in distinguishing between endometrial stromal sarcoma and uterine smooth muscle tumors: an immunohistochemical comparison of 34 cases. 54 61
11353058 2001
17
Effects of interleukin-4 on the in-vitro production of cytokines by human endometrial stromal cells. 61 54
11228246 2001
18
Estrogen receptor expression in an endometrial stromal sarcoma after tamoxifen therapy. 61 54
11874071 2001
19
Effects of interferon-gamma on cytokine production by endometrial stromal cells. 61 54
9806291 1998
20
Steroid hormone receptors in endometrial stromal sarcomas. A biochemical and immunohistochemical study. 61 54
1371902 1992
21
Endometrial stromal sarcoma expression of estrogen receptors, progesterone receptors and estrogen-induced srp27 (24K) suggests hormone responsiveness. 61 54
1562530 1992
22
Myogenous phenotype of epithelial-like areas in endometrial stromal sarcomas. 54 61
2001155 1991
23
Immunohistochemistry of endometrial stromal sarcoma. 61 54
1706303 1991
24
A novel MBTD1-PHF1 gene fusion in endometrial stromal sarcoma: A case report and literature review. 61
32237188 2020
25
The Many Faces of Endometriosis-Related Neoplasms in the Same Patient: A Brief Report. 61
32570258 2020
26
Gene of the month: BCOR. 61
32161069 2020
27
Effect of bilateral salpingo-oophorectomy on the overall survival of premenopausal patients with stage I low-grade endometrial stromal sarcoma; a National Cancer Database analysis. 61
32354469 2020
28
A nomogram for predicting overall survival in patients with low-grade endometrial stromal sarcoma: A population-based analysis. 61
32558385 2020
29
BCOR Expression in Mullerian Adenosarcoma: A Potential Diagnostic Pitfall. 61
32011345 2020
30
Uterine Endometrial Stromal Tumors With Limited Infiltration: First Report of a Case Series Indicating Potential for Malignant Behavior. 61
30807369 2020
31
Genomic profiling of BCOR-rearranged uterine sarcomas reveals novel gene fusion partners, frequent CDK4 amplification and CDKN2A loss. 61
32156473 2020
32
A Phase 1b Dose-Escalation Trial of NC-6300 (Nanoparticle Epirubicin) in Patients with Advanced Solid Tumors or Advanced, Metastatic, or Unresectable Soft Tissue Sarcoma. 61
32381487 2020
33
Primary colonic extrauterine endometrial stromal sarcoma: A case and review of the literature. 61
32382649 2020
34
Development of endometrial stromal sarcoma in a patient undergoing in vitro fertilization: A case report. 61
32123718 2020
35
Primary sarcoma of the cervix: an analysis of patient and tumor characteristics, treatment patterns, and outcomes. 61
31912680 2020
36
A retrospective study of endometrial stromal sarcoma: an institutional review. 61
32400756 2020
37
The clinical, morphological, and genetic heterogeneity of endometrial stromal sarcoma. 61
31993772 2020
38
Long-term impact of lymphadenectomies in patients with low-grade, early-stage uterine endometrial stroma sarcoma. 61
32022392 2020
39
The value of clinical parameters combined with magnetic resonance imaging (MRI) features for preoperatively distinguishing different subtypes of uterine sarcomas: An observational study (STROBE compliant). 61
32311989 2020
40
High-grade transformation of low-grade endometrial stromal sarcomas lacking YWHAE and BCOR genetic abnormalities. 61
32317704 2020
41
ZC3H7B-BCOR high-grade endometrial stromal sarcoma may present as myoma nascens with cytoplasmic signet ring cell change. 61
31938824 2020
42
Endometrial stromal sarcomas with BCOR-rearrangement harbor MDM2 amplifications. 61
32352245 2020
43
Genomic Landscape of Uterine Sarcomas Defined Through Prospective Clinical Sequencing. 61
32299819 2020
44
Natural Pregnancy and Successful Delivery in A Woman with Endometrial Stromal Sarcoma After Sequential Therapy with Heavy Dosage of Chinese Medicine: A Case Report. 61
32146593 2020
45
Uterine tumor resembling ovarian sex cord tumor: A series of six cases displaying varied histopathological patterns and clinical profiles. 61
32108635 2020
46
Right heart masses in a patient with endometrial stromal sarcoma. 61
31074021 2020
47
A Real-World Study on Diagnosis and Treatment of Uterine Sarcoma in Western China. 61
32015676 2020
48
Recurrent endometrial stromal sarcoma in the abdominal wall following a total hysterectomy: A rare case report. 61
32197223 2020
49
Evaluation of treatment results and prognostic factors of uterine sarcoma: A single-center experience. 61
31517774 2020
50
Collision Tumor of Endometrial Large Cell Neuroendocrine Carcinoma and Low-Grade Endometrial Stromal Sarcoma: A Case Report and Review of the Literature. 61
31992096 2020

Variations for Endometrial Stromal Sarcoma

Expression for Endometrial Stromal Sarcoma

Search GEO for disease gene expression data for Endometrial Stromal Sarcoma.

Pathways for Endometrial Stromal Sarcoma

Pathways related to Endometrial Stromal Sarcoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.19 VIM DES ACTC1
2 10.33 YWHAE ESR1 BCOR
3 9.56 ESR1 CYP19A1

GO Terms for Endometrial Stromal Sarcoma

Cellular components related to Endometrial Stromal Sarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 nucleus GO:0005634 9.47 ZC3H7B YWHAE VIM SUZ12 PHF1 PGR
2 ESC/E(Z) complex GO:0035098 8.96 SUZ12 PHF1

Biological processes related to Endometrial Stromal Sarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 uterus development GO:0060065 9.37 ESR1 CYP19A1
2 chromatin organization GO:0006325 9.35 SUZ12 PHF1 MEAF6 MBTD1 BCOR
3 androgen metabolic process GO:0008209 9.32 ESR1 CYP19A1
4 positive regulation of phospholipase C activity GO:0010863 9.26 KIT ESR1
5 negative regulation of histone H3-K27 methylation GO:0061086 9.16 PHF1 EZHIP
6 muscle filament sliding GO:0030049 8.8 VIM DES ACTC1

Molecular functions related to Endometrial Stromal Sarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 methylated histone binding GO:0035064 8.8 SUZ12 PHF1 MBTD1

Sources for Endometrial Stromal Sarcoma

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