MCID: END031
MIFTS: 52

Endometrial Stromal Sarcoma

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 spindle cell sarcoma and endometriosis. 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 Ifosfamide and Doxorubicin have been mentioned in the context of this disorder. Affiliated tissues include uterus, ovary and smooth muscle, 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 197)
# Related Disease Score Top Affiliating Genes
1 spindle cell sarcoma 32.0 ACTC1 DES VIM
2 endometriosis 31.8 CYP19A1 ESR1 PGR
3 endometrial stromal nodule 31.7 BCOR CYP19A1 JAZF1 MEAF6 MME NUTM2A
4 adenomyosis 31.1 CYP19A1 ESR1 MME PGR
5 leiomyosarcoma 31.1 ACTC1 DES KIT MB PGR VIM
6 myoma 31.0 CYP19A1 ESR1 PGR
7 lymphangioleiomyomatosis 31.0 DES ESR1 PGR
8 intravenous leiomyomatosis 31.0 DES ESR1 MME
9 uterine carcinosarcoma 30.9 KIT MME PGR
10 carcinosarcoma 30.8 ACTC1 DES KIT MME PGR VIM
11 endometriosis of ovary 30.7 CYP19A1 ESR1 PGR
12 leiomyoma 30.7 ACTC1 CYP19A1 DES ESR1 KIT MME
13 uterine sarcoma 30.7 CYP19A1 DES ESR1 KIT MB MME
14 hemangiopericytoma, malignant 30.7 ACTC1 DES VIM
15 smooth muscle tumor 30.6 ACTC1 DES ESR1 KIT MME PGR
16 endometrial adenocarcinoma 30.6 ESR1 MALAT1 PGR
17 adenofibroma 30.5 ACTC1 INHA MME VIM
18 myxoid leiomyosarcoma 30.5 ACTC1 BCOR MME PGR YWHAE
19 endometrial cancer 30.4 CCND1 CYP19A1 ESR1 KIT MME PGR
20 renal cell carcinoma, nonpapillary 30.3 KIT MALAT1 MME VIM
21 endometrial stromal tumor 30.0 BCOR JAZF1 MEAF6 MME NUTM2A PGR
22 vaginal endometrial stromal sarcoma 12.6
23 uterine corpus endometrial stromal sarcoma 12.4
24 cervical endometrial stromal sarcoma 12.4
25 empty sella syndrome 11.8
26 sarcoma 11.3
27 euthyroid sick syndrome 11.1
28 vestibular gland benign neoplasm 10.6 ESR1 PGR
29 bartholin's gland adenoma 10.6 ESR1 PGR
30 myofibroma 10.6 DES MB
31 vulvar syringoma 10.5 ESR1 PGR
32 lung leiomyoma 10.5 ESR1 PGR
33 glassy cell carcinoma of the cervix 10.5 ESR1 PGR
34 infantile digital fibromatosis 10.5 DES VIM
35 breast myofibroblastoma 10.5 ESR1 MME
36 vulvar benign neoplasm 10.5 ESR1 PGR
37 lymphangiectasis 10.5 ACTC1 VIM
38 lymphangiomatosis 10.5 DES PGR VIM
39 trigonitis 10.5 ESR1 PGR
40 vulvar leiomyoma 10.5 ESR1 PGR
41 epulis 10.5 DES PGR VIM
42 progesterone-receptor positive breast cancer 10.5 CYP19A1 ESR1 PGR
43 calcifying aponeurotic fibroma 10.5 ACTC1 PGR
44 fibroblastic rheumatism 10.5 ACTC1 DES
45 gender identity disorder 10.5 CYP19A1 ESR1 PGR
46 estrogen excess 10.5 CYP19A1 ESR1 PGR
47 mammographic density 10.5 CYP19A1 ESR1 PGR
48 angiolipoma 10.5 ACTC1 DES VIM
49 mesenchymoma 10.5 DES MB
50 cervical carcinosarcoma 10.5 ESR1 MME PGR

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 76)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Ifosfamide Approved Phase 3,Phase 2 3778-73-2 3690
2
Doxorubicin Approved, Investigational Phase 3,Phase 2 23214-92-8 31703
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 Antineoplastic Agents, Alkylating Phase 3,Phase 2
9 Alkylating Agents Phase 3,Phase 2
10
Isophosphamide mustard Phase 3,Phase 2 0
11 Anti-Bacterial Agents Phase 3,Phase 2
12 Antibiotics, Antitubercular Phase 3,Phase 2
13 Topoisomerase Inhibitors Phase 3,Phase 2
14 Gastrointestinal Agents Phase 3,Phase 2
15 Histamine Antagonists Phase 3
16 Neurotransmitter Agents Phase 3
17 Serotonin Agents Phase 3
18 Serotonin Antagonists Phase 3
19 Anti-Allergic Agents Phase 3
20 Antipruritics Phase 3
21 Dermatologic Agents Phase 3
22 Histamine H1 Antagonists Phase 3
23
Histamine Phosphate Phase 3 51-74-1 65513
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
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
28
Hydrocortisone Approved, Vet_approved Phase 2 50-23-7 5754
29
Hydrocortisone acetate Approved, Vet_approved Phase 2 50-03-3
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
Exemestane Approved, Investigational Phase 2 107868-30-4 60198
35
Anastrozole Approved, Investigational Phase 2 120511-73-1 2187
36
Letrozole Approved, Investigational Phase 2 112809-51-5 3902
37
Fulvestrant Approved, Investigational Phase 2 129453-61-8 17756771 104741
38
nivolumab Approved Phase 2 946414-94-4
39
Ipilimumab Approved Phase 2 477202-00-9
40
Saracatinib Investigational Phase 2 379231-04-6
41
Trebananib Investigational Phase 2 894356-79-7
42 Hydrocortisone 17-butyrate 21-propionate Phase 2
43 Antineoplastic Agents, Hormonal Phase 2
44 Antiemetics Phase 2
45 glucocorticoids Phase 2
46 HIV Protease Inhibitors Phase 2
47 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2
48 BB 1101 Phase 2
49 Hydrocortisone hemisuccinate Phase 2
50 Hormones Phase 2

Interventional clinical trials:

(show all 24)
# 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 FUlvestrant in Gynecological Cancers That Are Potentially Hormone Sensitive: the FUCHSia Study Recruiting NCT03926936 Phase 2 Fulvestrant
21 A Study of Nivolumab in Selected Uterine Cancer Patients Recruiting NCT03241745 Phase 2 Nivolumab
22 IRCI Gynae Sarcomas, High Grade Uterine Sarcoma Recruiting NCT01979393 Phase 2 Cabozantinib;Placebo
23 A Phase II of Nivolumab Plus Ipilimumab in Non-resectable Sarcoma and Endometrial Carcinoma Not yet recruiting NCT02982486 Phase 2 Ipilimumab;Nivolumab
24 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, Ovary, Smooth Muscle, Heart, Colon, Cervix, Kidney

Publications for Endometrial Stromal Sarcoma

Articles related to Endometrial Stromal Sarcoma:

(show top 50) (show all 677)
# Title Authors Year
1
Extrauterine, Extragenital Endometrial Stromal Sarcoma in the Absence of Endometriosis: A Rare Entity. ( 29210799 )
2019
2
Adjuvant Hormonal Therapy for Low-Grade Endometrial Stromal Sarcoma. ( 29843577 )
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
Localized high grade endometrial stromal sarcoma and localized undifferentiated uterine sarcoma: a retrospective series of the French Sarcoma Group. ( 30772825 )
2019
7
Tumour lysis syndrome in a patient with undifferentiated endometrial stromal sarcoma. ( 30834285 )
2019
8
Treatment of uterine high-grade endometrial stromal sarcoma with apatinib combined with chemotherapy: A case report. ( 30921232 )
2019
9
The routine use of progestins might be a good choice for patients with recurrent low-grade endometrial stromal sarcoma after definite surgery. ( 30932937 )
2019
10
Hormone therapy and low-grade endometrial stromal sarcoma. ( 30932941 )
2019
11
Endometrial Stromal Sarcoma: Case Series with Emphasis on Gross Features. ( 30948880 )
2019
12
Survival outcomes of different treatment modalities in patients with low-grade endometrial stromal sarcoma. ( 30985313 )
2019
13
Reply to "The routine use of progestins might be a good choice for patients with recurrent low-grade endometrial stromal sarcoma after definite surgery ". ( 31008813 )
2019
14
Metastatic low-grade endometrial stromal sarcoma of uterus presenting as a primary pancreatic tumor: case presentation and literature review. ( 31010432 )
2019
15
Hormone therapy following surgery in low-grade endometrial stromal sarcoma: Is it related to a decrease in recurrence rate? ( 31058712 )
2019
16
Right heart masses in a patient with endometrial stromal sarcoma. ( 31074021 )
2019
17
Low-grade endometrial stromal sarcoma with a novel MEAF6-SUZ12 fusion. ( 31101969 )
2019
18
Is greater antibiotic therapy prior to ESS associated with differences in surgical outcomes in CRS? ( 30582166 )
2019
19
Endoscopic Spinal Surgery (ESS): Prepare for a Happy 100-Year-Old! ( 30943701 )
2019
20
YWHAE Rearrangement in a Purely Conventional Low-grade Endometrial Stromal Sarcoma that Transformed Over Time to High-grade Sarcoma: Importance of Molecular Testing. ( 28863072 )
2018
21
A remarkable response to pazopanib, despite recurrent liver toxicity, in a patient with a high grade endometrial stromal sarcoma, a case report. ( 29357824 )
2018
22
Immunohistochemical Expression of Different Subtypes of Cytokeratins by Endometrial Stromal Sarcoma. ( 29406332 )
2018
23
Impact of oophorectomy and hormone suppression in low grade endometrial stromal sarcoma: A multicenter review. ( 29534832 )
2018
24
Gene expression profiling of low-grade endometrial stromal sarcoma indicates fusion protein-mediated activation of the Wnt signaling pathway. ( 29544705 )
2018
25
Endometrial Stromal Sarcoma With Hyalinizing Giant Rosettes, Mimicking Low-Grade Fibromyxoid Sarcoma. ( 29623744 )
2018
26
Molecular biomarkers for uterine leiomyosarcoma and endometrial stromal sarcoma. ( 29660202 )
2018
27
Identification of an EPC2-PHF1 fusion transcript in low-grade endometrial stromal sarcoma. ( 29721194 )
2018
28
Morphological and immunohistochemical diversity of endometrial stromal sarcoma in rats. ( 29749998 )
2018
29
Hepatic endometrial stromal sarcoma. ( 29843927 )
2018
30
Investigation of hormone receptor expression and its prognostic value in endometrial stromal sarcoma. ( 29869299 )
2018
31
Treatment of low-grade endometrial stromal sarcoma in a nulligravid woman. ( 29887484 )
2018
32
Recurrence after fertility-preserving surgery for low-grade endometrial stromal sarcoma. ( 29974555 )
2018
33
Next-generation Sequencing of an Ovarian Spindle Cell Tumor Identified an Ovarian Low-grade Endometrial Stromal Sarcoma: A Rare Entity. ( 30028354 )
2018
34
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
35
Novel EPC1 gene fusions in endometrial stromal sarcoma. ( 30144186 )
2018
36
Endometrial Stromal Sarcoma Recurrence in the Caecum. ( 30174981 )
2018
37
YWHAE-NUTM2A/B Translocated High-grade Endometrial Stromal Sarcoma Commonly Expresses CD56 and CD99. ( 30252726 )
2018
38
Low-Grade Endometrial Stromal Sarcoma in a Postmenopausal Woman with Third-Degree Uterovaginal Prolapse: A Rare Case with Review of the Literature. ( 30294191 )
2018
39
Low-Grade Endometrial Stromal Sarcoma - a Review. ( 30317238 )
2018
40
Endometrial Stromal Sarcoma Arising from Endometrial Polyp: A Case Report. ( 30381725 )
2018
41
Low-grade endometrial stromal sarcoma combined with leiomyoma (stromomyoma): A bizarre tumor of the uterus in a premenopausal woman. ( 30476382 )
2018
42
Aromatase inhibitor therapy for endometrial stromal sarcoma - two-centre experience. ( 30508212 )
2018
43
Endometrial stromal sarcoma in a 27-year-old woman. Case report and literature review. ( 30534836 )
2018
44
Retroperitoneal low grade endometrial stromal sarcoma with florid endometrioid glandular differentiation: Cytologic-histologic correlation and differential diagnosis. ( 30611894 )
2018
45
A Case Report of High-Grade Endometrial Stromal Sarcoma: A Rare Cause of Abnormal Uterine Bleeding in a Young Woman. ( 30622826 )
2018
46
ESS: A Tool for Genome-Scale Quantification of Essentiality Score for Reaction/Genes in Constraint-Based Modeling. ( 30323767 )
2018
47
Suitability of the Epworth Sleepiness Scale (ESS) for Economic Evaluation: An Assessment of Its Convergent and Discriminant Validity. ( 27754703 )
2018
48
Is reproducibility inside the bag? Special issue fundamentals and applications of sonochemistry ESS-15. ( 28377103 )
2018
49
The ESS and replicator equation in matrix games under time constraints. ( 29332298 )
2018
50
Discovering the effect of nonlocal payoff calculation on the stabilty of ESS: Spatial patterns of Hawk-Dove game in metapopulations. ( 29355541 )
2018

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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