ESS
MCID: END031
MIFTS: 52

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 77 54 60 15 17 74
Ess 12 54
Endometrial Stromal Sarcoma, High Grade 12
Undifferentiated Endometrial Sarcoma 12
Stromal Sarcoma of the Corpus Uteri 60
Undifferentiated Stromal Sarcoma 74
Sarcoma, Endometrial Stromal 45
Sarcoma Endometrial Stromal 56

Classifications:



External Ids:

Disease Ontology 12 DOID:4226
MeSH 45 D018203
NCIt 51 C8973
SNOMED-CT 69 70555003
MESH via Orphanet 46 D018203
ICD10 via Orphanet 35 C54.1
UMLS via Orphanet 75 C0206630
Orphanet 60 ORPHA213711

Summaries for Endometrial Stromal Sarcoma

NIH Rare Diseases : 54 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 endometriosis 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 Regulation of Telomerase. The drugs Doxorubicin and Ifosfamide have been mentioned in the context of this disorder. Affiliated tissues include uterus, smooth muscle and colon, and related phenotypes are cardiovascular system and cellular

Disease Ontology : 12 An endometrial stromal tumor that has_material_basis in connective tissue.

Wikipedia : 77 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 192)
# Related Disease Score Top Affiliating Genes
1 endometriosis 31.8 CYP19A1 ESR1 PGR
2 endometrial stromal nodule 31.7 BCOR CYP19A1 JAZF1 MEAF6 MME NUTM2A
3 spindle cell sarcoma 31.6 ACTC1 DES VIM
4 leiomyosarcoma 31.2 ACTC1 DES KIT MB PGR VIM
5 adenomyosis 31.1 CYP19A1 ESR1 MME PGR
6 myoma 31.0 CYP19A1 ESR1 PGR
7 intravenous leiomyomatosis 31.0 DES ESR1 MME
8 uterine carcinosarcoma 30.9 KIT MME PGR
9 carcinosarcoma 30.9 ACTC1 DES KIT MME PGR VIM
10 lymphangioleiomyomatosis 30.8 DES ESR1 PGR
11 leiomyoma 30.7 ACTC1 CYP19A1 DES ESR1 KIT MME
12 uterine sarcoma 30.7 CYP19A1 DES ESR1 KIT MB MME
13 endometriosis of ovary 30.7 CYP19A1 ESR1 PGR
14 smooth muscle tumor 30.7 ACTC1 DES ESR1 KIT MME PGR
15 endometrial adenocarcinoma 30.6 ESR1 MALAT1 PGR
16 adenofibroma 30.5 ACTC1 INHA MME VIM
17 myxoid leiomyosarcoma 30.5 ACTC1 BCOR MME PGR YWHAE
18 endometrial cancer 30.4 CCND1 CYP19A1 ESR1 KIT MME PGR
19 renal cell carcinoma, nonpapillary 30.3 KIT MALAT1 MME VIM
20 endometrial stromal tumor 30.0 BCOR JAZF1 MEAF6 MME NUTM2A PGR
21 vaginal endometrial stromal sarcoma 12.5
22 uterine corpus endometrial stromal sarcoma 12.4
23 cervical endometrial stromal sarcoma 12.4
24 empty sella syndrome 11.7
25 sarcoma 11.3
26 euthyroid sick syndrome 11.1
27 myofibroma 10.6 DES MB
28 vestibular gland benign neoplasm 10.6 ESR1 PGR
29 bartholin's gland adenoma 10.6 ESR1 PGR
30 vulvar syringoma 10.6 ESR1 PGR
31 lung leiomyoma 10.6 ESR1 PGR
32 glassy cell carcinoma of the cervix 10.5 ESR1 PGR
33 infantile digital fibromatosis 10.5 DES VIM
34 breast myofibroblastoma 10.5 ESR1 MME
35 lymphangiomatosis 10.5 DES PGR VIM
36 lymphangiectasis 10.5 ACTC1 VIM
37 vulvar benign neoplasm 10.5 ESR1 PGR
38 trigonitis 10.5 ESR1 PGR
39 vulvar leiomyoma 10.5 ESR1 PGR
40 epulis 10.5 DES PGR VIM
41 progesterone-receptor positive breast cancer 10.5 CYP19A1 ESR1 PGR
42 calcifying aponeurotic fibroma 10.5 ACTC1 PGR
43 gender identity disorder 10.5 CYP19A1 ESR1 PGR
44 fibroblastic rheumatism 10.5 ACTC1 DES
45 estrogen excess 10.5 CYP19A1 ESR1 PGR
46 mammographic density 10.5 CYP19A1 ESR1 PGR
47 angiolipoma 10.5 ACTC1 DES VIM
48 mesenchymoma 10.5 DES MB
49 cervical carcinosarcoma 10.5 ESR1 MME PGR
50 endometrial mucinous adenocarcinoma 10.5 ESR1 PGR VIM

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:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.18 ACTC1 BCOR CCND1 CYP19A1 DES ESR1
2 cellular MP:0005384 10.15 ACTC1 BCOR CCND1 CYP19A1 DES ESR1
3 homeostasis/metabolism MP:0005376 10.03 ACTC1 CCND1 CYP19A1 DES ESR1 INHA
4 hematopoietic system MP:0005397 10.02 BCOR CCND1 CYP19A1 ESR1 INHA KIT
5 mortality/aging MP:0010768 9.93 ACTC1 BCOR CCND1 DES ESR1 INHA
6 muscle MP:0005369 9.61 ACTC1 CYP19A1 DES ESR1 KIT MB
7 neoplasm MP:0002006 9.1 CCND1 ESR1 INHA KIT PGR SUZ12

Drugs & Therapeutics for Endometrial Stromal Sarcoma

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

(show top 50) (show all 75)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Doxorubicin Approved, Investigational Phase 3,Phase 2 23214-92-8 31703
2
Ifosfamide Approved Phase 3,Phase 2 3778-73-2 3690
3
Lenograstim Approved, Investigational Phase 3 135968-09-1
4
Mechlorethamine Approved, Investigational Phase 3 51-75-2 4033
5
Histamine Approved, Investigational Phase 3 51-45-6 774
6
Cyproheptadine Approved Phase 3 129-03-3 2913
7
Doxil Approved June 1999 Phase 3,Phase 2 31703
8 Alkylating Agents Phase 3,Phase 2
9 Antineoplastic Agents, Alkylating Phase 3,Phase 2
10 Topoisomerase Inhibitors Phase 3,Phase 2
11 Anti-Bacterial Agents Phase 3,Phase 2
12 Antibiotics, Antitubercular Phase 3,Phase 2
13
Isophosphamide mustard Phase 3,Phase 2 0
14 Gastrointestinal Agents Phase 3,Phase 2
15 Dermatologic Agents Phase 3
16 Serotonin Antagonists Phase 3
17 Anti-Allergic Agents Phase 3
18 Neurotransmitter Agents Phase 3
19 Histamine Antagonists Phase 3
20 Antipruritics Phase 3
21 Histamine H1 Antagonists Phase 3
22
Histamine Phosphate Phase 3 51-74-1 65513
23 Serotonin Agents Phase 3
24
Serotonin Investigational, Nutraceutical Phase 3 50-67-9 5202
25
Temozolomide Approved, Investigational Phase 2 85622-93-1 5394
26
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 2 1177-87-3
27
Hydrocortisone acetate Approved, Vet_approved Phase 2 50-03-3
28
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
29
Hydrocortisone Approved, Vet_approved Phase 2 50-23-7 5754
30
Mifepristone Approved, Investigational Phase 2 84371-65-3 55245
31
Sunitinib Approved, Investigational Phase 2 557795-19-4, 341031-54-7 5329102
32
Trabectedin Approved, Investigational Phase 2 114899-77-3 108150
33
Paclitaxel Approved, Vet_approved Phase 2 33069-62-4 36314
34
Letrozole Approved, Investigational Phase 2 112809-51-5 3902
35
Exemestane Approved, Investigational Phase 2 107868-30-4 60198
36
Anastrozole Approved, Investigational Phase 2 120511-73-1 2187
37
nivolumab Approved Phase 2 946414-94-4
38
Ipilimumab Approved Phase 2 477202-00-9
39
Saracatinib Investigational Phase 2 379231-04-6
40
Trebananib Investigational Phase 2 894356-79-7
41 Autonomic Agents Phase 2
42 Hormone Antagonists Phase 2
43 HIV Protease Inhibitors Phase 2
44 glucocorticoids Phase 2
45 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2
46 Antineoplastic Agents, Hormonal Phase 2
47 Hormones Phase 2
48 Anti-Inflammatory Agents Phase 2
49 Peripheral Nervous System Agents Phase 2
50
protease inhibitors Phase 2

Interventional clinical trials:

(show all 23)
# Name Status NCT ID Phase Drugs
1 Radiation Therapy or No Further Treatment Following Surgery in Treating Patients With Cancer of the Uterus Completed NCT00002459 Phase 3
2 Surgery With or Without Combination Chemotherapy in Treating Patients With Lung Metastases From Soft Tissue Sarcoma Completed NCT00002764 Phase 3 doxorubicin hydrochloride;ifosfamide
3 Surgery With or Without Chemotherapy in Treating Patients With Soft Tissue Sarcoma Completed NCT00002641 Phase 3 doxorubicin hydrochloride;ifosfamide;isolated perfusion
4 Cyproheptadine in Preventing Weight Loss in Children Receiving Chemotherapy for Cancer Terminated NCT01132547 Phase 3 cyproheptadine hydrochloride
5 Temozolomide in Treating Patients With Advanced Soft Tissue Sarcoma Unknown status NCT00003718 Phase 2 temozolomide
6 Medical Safety Follow-up Study for Patients Who Received More Than 28 Days of Total Exposure to BGG492 Completed NCT02150213 Phase 2 BGG492
7 Mifepristone for Patients With Endometrial Cancer and LGESS Completed NCT00505739 Phase 2 Mifepristone
8 Chemotherapy in Treating Patients With Sarcoma of the Uterus Completed NCT00005643 Phase 2 pegylated liposomal doxorubicin hydrochloride
9 AZD0530 in Treating Patients With Recurrent Locally Advanced or Metastatic Soft Tissue Sarcoma Completed NCT00659360 Phase 2 saracatinib
10 BI 2536 in Treating Patients With Recurrent or Metastatic Solid Tumors Completed NCT00526149 Phase 2 BI 2536
11 Sunitinib in Treating Patients With Metastatic, Locally Advanced, or Locally Recurrent Sarcomas Completed NCT00474994 Phase 2 sunitinib malate
12 Ecteinascidin 743 in Treating Patients With Advanced Soft Tissue Sarcoma Completed NCT00003939 Phase 2 trabectedin
13 STI571 in Treating Patients With Recurrent or Refractory Soft Tissue Sarcoma Completed NCT00006357 Phase 1, Phase 2 imatinib mesylate
14 Combination Chemotherapy in Treating Patients With Endometrial Cancer, Fallopian Tube Cancer, or Sarcoma of the Female Reproductive Tract Completed NCT00003334 Phase 2 paclitaxel;pegylated liposomal doxorubicin hydrochloride
15 Perifosine in Treating Patients With Metastatic or Locally Advanced Soft Tissue Sarcoma Completed NCT00053794 Phase 2 perifosine
16 Thalidomide in Treating Patients With Gynecologic Sarcomas Completed NCT00006005 Phase 2 thalidomide
17 Trebananib in Treating Patients With Persistent or Recurrent Endometrial Cancer Completed NCT01210222 Phase 2
18 Phase II Study of Imatinib Mesylate in Patients With Life Threatening Malignant Rare Diseases Completed NCT00154388 Phase 2 Imatinib mesylate
19 Evaluation of Clinical Impact of Interruption VS Maintenance of AI in Patients With Locally Advanced/ Metastatic Low Grade Endometrial Stromal Sarcoma (LGESS) Recruiting NCT03624244 Phase 2 Aromatase Inhibitors
20 A Study of Nivolumab in Selected Uterine Cancer Patients Recruiting NCT03241745 Phase 2 Nivolumab
21 IRCI Gynae Sarcomas, High Grade Uterine Sarcoma Recruiting NCT01979393 Phase 2 Cabozantinib;Placebo
22 A Phase II of Nivolumab Plus Ipilimumab in Non-resectable Sarcoma and Endometrial Carcinoma Not yet recruiting NCT02982486 Phase 2 Ipilimumab;Nivolumab
23 Collecting Tumor Samples From Patients With Gynecological Tumors Completed NCT00897442

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:

42
Uterus, Smooth Muscle, Colon, Ovary, Breast, Testes, Liver

Publications for Endometrial Stromal Sarcoma

Articles related to Endometrial Stromal Sarcoma:

(show top 50) (show all 661)
# Title Authors Year
1
Localized high grade endometrial stromal sarcoma and localized undifferentiated uterine sarcoma: a retrospective series of the French Sarcoma Group. ( 30772825 )
2019
2
Tumour lysis syndrome in a patient with undifferentiated endometrial stromal sarcoma. ( 30834285 )
2019
3
Ovarian preservation for low-grade endometrial stromal sarcoma: a systematic review of the literature and meta-analysis. ( 30640694 )
2019
4
High-grade endometrial stromal sarcoma: a retrospective study of factors influencing prognosis. ( 30697075 )
2019
5
Metastatic low-grade endometrial stromal sarcoma with sex cord and smooth muscle differentiation: A case report. ( 30705899 )
2019
6
Is greater antibiotic therapy prior to ESS associated with differences in surgical outcomes in CRS? ( 30582166 )
2019
7
Hepatic endometrial stromal sarcoma. ( 29843927 )
2018
8
A remarkable response to pazopanib, despite recurrent liver toxicity, in a patient with a high grade endometrial stromal sarcoma, a case report. ( 29357824 )
2018
9
Recurrence after fertility-preserving surgery for low-grade endometrial stromal sarcoma. ( 29974555 )
2018
10
Endometrial Stromal Sarcoma With Hyalinizing Giant Rosettes, Mimicking Low-Grade Fibromyxoid Sarcoma. ( 29623744 )
2018
11
Adjuvant Hormonal Therapy for Low-Grade Endometrial Stromal Sarcoma. ( 29843577 )
2018
12
Morphological and immunohistochemical diversity of endometrial stromal sarcoma in rats. ( 29749998 )
2018
13
Gene expression profiling of low-grade endometrial stromal sarcoma indicates fusion protein-mediated activation of the Wnt signaling pathway. ( 29544705 )
2018
14
Investigation of hormone receptor expression and its prognostic value in endometrial stromal sarcoma. ( 29869299 )
2018
15
Impact of oophorectomy and hormone suppression in low grade endometrial stromal sarcoma: A multicenter review. ( 29534832 )
2018
16
Molecular biomarkers for uterine leiomyosarcoma and endometrial stromal sarcoma. ( 29660202 )
2018
17
Immunohistochemical Expression of Different Subtypes of Cytokeratins by Endometrial Stromal Sarcoma. ( 29406332 )
2018
18
Treatment of low-grade endometrial stromal sarcoma in a nulligravid woman. ( 29887484 )
2018
19
Identification of an <i>EPC2-PHF1</i> fusion transcript in low-grade endometrial stromal sarcoma. ( 29721194 )
2018
20
Next-generation Sequencing of an Ovarian Spindle Cell Tumor Identified an Ovarian Low-grade Endometrial Stromal Sarcoma: A Rare Entity. ( 30028354 )
2018
21
Low-grade endometrial stromal sarcoma presenting as multiple pulmonary nodules: A potential pitfall in fine needle aspiration and core biopsy specimens - A Cytological - Pathological Correlation. ( 30055523 )
2018
22
Novel EPC1 gene fusions in endometrial stromal sarcoma. ( 30144186 )
2018
23
Endometrial Stromal Sarcoma Recurrence in the Caecum. ( 30174981 )
2018
24
YWHAE-NUTM2A/B Translocated High-grade Endometrial Stromal Sarcoma Commonly Expresses CD56 and CD99. ( 30252726 )
2018
25
Low-Grade Endometrial Stromal Sarcoma in a Postmenopausal Woman with Third-Degree Uterovaginal Prolapse: A Rare Case with Review of the Literature. ( 30294191 )
2018
26
Low-Grade Endometrial Stromal Sarcoma - a Review. ( 30317238 )
2018
27
Endometrial Stromal Sarcoma Arising from Endometrial Polyp: A Case Report. ( 30381725 )
2018
28
Low-grade endometrial stromal sarcoma combined with leiomyoma (stromomyoma): A bizarre tumor of the uterus in a premenopausal woman. ( 30476382 )
2018
29
Aromatase inhibitor therapy for endometrial stromal sarcoma - two-centre experience. ( 30508212 )
2018
30
Endometrial stromal sarcoma in a 27-year-old woman. Case report and literature review. ( 30534836 )
2018
31
Retroperitoneal low grade endometrial stromal sarcoma with florid endometrioid glandular differentiation: Cytologic-histologic correlation and differential diagnosis. ( 30611894 )
2018
32
A Case Report of High-Grade Endometrial Stromal Sarcoma: A Rare Cause of Abnormal Uterine Bleeding in a Young Woman. ( 30622826 )
2018
33
Is reproducibility inside the bag? Special issue fundamentals and applications of sonochemistry ESS-15. ( 28377103 )
2018
34
The ESS and replicator equation in matrix games under time constraints. ( 29332298 )
2018
35
Discovering the effect of nonlocal payoff calculation on the stabilty of ESS: Spatial patterns of Hawk-Dove game in metapopulations. ( 29355541 )
2018
36
Koskinen et al. Long-term follow-up after ESS and balloon sinoplasty: comparison of symptom reduction and patient satisfaction. Acta Oto-Laryngol 2016; 136: 532-536. ( 29532720 )
2018
37
Optimization of moderators and beam extraction at the ESS. ( 29657564 )
2018
38
Sleep-disordered breathing in patients with cardiovascular diseases cannot be detected by ESS, STOP-BANG, and Berlin questionnaires. ( 29845331 )
2018
39
Effect of ESS on Olfactory Threshold of Patients with CRS without Nasal Polyps. ( 29993224 )
2018
40
The Emergency Surgery Score (ESS) accurately predicts the occurrence of postoperative complications in emergency surgery patients: A reply. ( 30036260 )
2018
41
Can the emergency surgery score (ESS) be used as a triage tool predicting the postoperative need for an ICU admission? ( 30172358 )
2018
42
ESS: A Tool for Genome-Scale Quantification of Essentiality Score for Reaction/Genes in Constraint-Based Modeling. ( 30323767 )
2018
43
Suitability of the Epworth Sleepiness Scale (ESS) for Economic Evaluation: An Assessment of Its Convergent and Discriminant Validity. ( 27754703 )
2018
44
BCOR is a robust diagnostic immunohistochemical marker of genetically diverse high-grade endometrial stromal sarcoma, including tumors exhibiting variant morphology. ( 28621321 )
2017
45
Magnetic resonance imaging features of endometrial stromal sarcoma: a case description. ( 28275572 )
2017
46
Androgen Receptor Expression in Endometrial Stromal Sarcoma: Correlation With Clinicopathologic Features. ( 28114189 )
2017
47
A recurrent endometrial stromal sarcoma harbors the novel fusion JAZF1-BCORL1. ( 28331900 )
2017
48
Cardiac metastatic endometrial stromal sarcoma 17 years after hysterectomy. ( 28967156 )
2017
49
Oncogenic HrasG12V expression plus knockdown of Cdkn2a using ecotropic lentiviral vectors induces high-grade endometrial stromal sarcoma. ( 28982163 )
2017
50
Primary low-grade endometrial stromal sarcoma arising in the vagina: report of an unusual case and literature review. ( 29218216 )
2017

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 11.36 ACTC1 DES VIM
2 11.25 CCND1 ESR1 YWHAE
3 10.66 BCOR ESR1 YWHAE
4 10.3 CYP19A1 INHA PGR
5 9.76 CYP19A1 ESR1

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 ESC/E(Z) complex GO:0035098 8.62 PHF1 SUZ12

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

# Name GO ID Score Top Affiliating Genes
1 male gonad development GO:0008584 9.33 ESR1 INHA KIT
2 androgen metabolic process GO:0008209 9.32 CYP19A1 ESR1
3 mammary gland alveolus development GO:0060749 9.26 CCND1 ESR1
4 positive regulation of phospholipase C activity GO:0010863 8.96 ESR1 KIT
5 muscle filament sliding GO:0030049 8.8 ACTC1 DES VIM

Sources for Endometrial Stromal Sarcoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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