MCID: END031
MIFTS: 48

Endometrial Stromal Sarcoma

Categories: Rare diseases, Reproductive diseases, Cancer diseases

Aliases & Classifications for Endometrial Stromal Sarcoma

MalaCards integrated aliases for Endometrial Stromal Sarcoma:

Name: Endometrial Stromal Sarcoma 12 50 56 14 69
Ess 12 50
Endometrial Stromal Sarcoma, High Grade 12
Undifferentiated Endometrial Sarcoma 12
Stromal Sarcoma of the Corpus Uteri 56
Undifferentiated Stromal Sarcoma 69
Sarcoma, Endometrial Stromal 42
Sarcoma Endometrial Stromal 52

Classifications:



External Ids:

Disease Ontology 12 DOID:4226
MeSH 42 D018203
NCIt 47 C8973
SNOMED-CT 64 699356008 70555003
Orphanet 56 ORPHA213711
MESH via Orphanet 43 D018203
UMLS via Orphanet 70 C0206630
ICD10 via Orphanet 34 C54.1
UMLS 69 C0206630

Summaries for Endometrial Stromal Sarcoma

NIH Rare Diseases : 50 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. last updated: 2/15/2016

MalaCards based summary : Endometrial Stromal Sarcoma, also known as ess, is related to myoma and myxoid leiomyosarcoma. An important gene associated with Endometrial Stromal Sarcoma is JAZF1 (JAZF Zinc Finger 1), and among its related pathways/superpathways are Integrated Breast Cancer Pathway and Striated Muscle Contraction. The drugs Doxorubicin and Ifosfamide 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

Wikipedia : 72 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 205)
id Related Disease Score Top Affiliating Genes
1 myoma 31.3 ACTC1 DES VIM
2 myxoid leiomyosarcoma 31.1 ACTC1 DES VIM
3 cervical endometrial stromal sarcoma 12.1
4 uterine corpus endometrial stromal sarcoma 12.1
5 vaginal endometrial stromal sarcoma 12.1
6 empty sella syndrome 11.5
7 endometrial stromal nodule 11.4
8 sarcoma 11.1
9 bartholin's gland benign neoplasm 11.0 ESR1 PGR
10 infantile scoliosis 11.0 DES VIM
11 lymphocytic hypophysitis 11.0 ACTC1 VIM
12 gallbladder leiomyoma 11.0 ESR1 PGR
13 glaucoma, hereditary 11.0 ESR1 PGR
14 lymphocytic infiltrate of jessner 11.0 DES PGR VIM
15 breast angiomatosis 11.0 ESR1 MME
16 pineal region yolk sac tumor 11.0 DES PGR VIM
17 fallopian tube benign neoplasm 11.0 ESR1 PGR
18 blastema predominant kidney wilms' tumor 11.0 PGR TP53
19 her2-receptor positive breast cancer 11.0 CYP19A1 ESR1 PGR
20 paranasal sinus disease 11.0 ESR1 PGR
21 chronic salpingitis 11.0 DES ESR1 MME
22 osteogenesis imperfecta 11.0 CYP19A1 ESR1 PGR
23 dartoic leiomyoma 11.0 ESR1 PGR
24 acute thyroiditis 11.0 CYP19A1 ESR1 PGR
25 aortic aneurysm 11.0 ACTC1 DES VIM
26 esophagus leiomyosarcoma 11.0 ACTC1 DES VIM
27 bladder colloid adenocarcinoma 11.0 ESR1 PGR VIM
28 perinatal jaundice due to hepatocellular damage 11.0 CYP19A1 ESR1 PGR
29 spleen angiosarcoma 11.0 ACTC1 DES VIM
30 telo2-related intellectual disability-neurodevelopmental disorder 11.0 ACTC1 DES
31 skin lipoma 11.0 ACTC1 DES VIM
32 mucin-rich endometrial endometrioid adenocarcinoma 11.0 DES PGR VIM
33 laryngeal neuroendocrine tumor 11.0 ACTC1 DES VIM
34 uniparental disomy of chromosome 11 11.0 ACTC1 DES VIM
35 vulvar squamous papilloma 10.9 DES ESR1 VIM
36 gastric signet ring cell adenocarcinoma 10.9 ACTC1 ESR1 VIM
37 esophagus sarcoma 10.9 CYP19A1 ESR1 PGR
38 follicular infundibulum tumor 10.9 ACTC1 DES VIM
39 uterine corpus apoplectic leiomyoma 10.9 ESR1 MME PGR
40 juvenile xanthogranuloma 10.9 ACTC1 DES VIM
41 fallopian tube papillary adenocarcinoma 10.9 ESR1 MME PGR
42 short-rib thoracic dysplasia 12 10.9 ACTC1 DES VIM
43 coronary restenosis 10.9 ACTC1 DES VIM
44 marfanoid habitus-inguinal hernia-advanced bone age syndrome 10.9 ACTC1 DES KIT
45 nephrogenic adenoma of urinary bladder 10.9 ACTC1 DES VIM
46 superficial basal cell carcinoma 10.9 ESR1 KIT PGR
47 lung leiomyosarcoma 10.9 JAZF1 KIT PHF1
48 multiple mitochondrial dysfunctions syndrome 2 10.9 ACTC1 DES VIM
49 central nervous system organ benign neoplasm 10.9 ESR1 PGR TP53
50 capillary disease 10.9 CYP19A1 ESR1 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:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.1 CCND1 CYP19A1 DES ESR1 INHA KIT
2 cellular MP:0005384 10 ACTC1 CCND1 CYP19A1 DES ESR1 KIT
3 muscle MP:0005369 9.81 ACTC1 CYP19A1 DES ESR1 KIT PGR
4 neoplasm MP:0002006 9.5 CCND1 ESR1 INHA KIT PGR SUZ12
5 reproductive system MP:0005389 9.28 INHA KIT PGR TP53 VIM YWHAE

Drugs & Therapeutics for Endometrial Stromal Sarcoma

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

(show top 50) (show all 67)
id 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 3778-73-2 3690
3
Lenograstim Approved Phase 3 135968-09-1
4
Mechlorethamine Approved Phase 3 51-75-2 4033
5
Cyproheptadine Approved Phase 3 129-03-3 2913
6
Histamine Approved, Investigational Phase 3 75614-87-8, 51-45-6 774
7
Doxil Approved June 1999 Phase 3,Phase 2 31703
8 Alkylating Agents Phase 3,Phase 2
9 Anti-Bacterial Agents Phase 3,Phase 2
10 Antibiotics, Antitubercular Phase 3,Phase 2
11 Isophosphamide mustard Phase 3
12 Topoisomerase Inhibitors Phase 3,Phase 2
13 Gastrointestinal Agents Phase 3,Phase 2
14 Anti-Allergic Agents Phase 3
15 Antipruritics Phase 3
16 Dermatologic Agents Phase 3
17 Histamine Antagonists Phase 3
18 Histamine H1 Antagonists Phase 3
19
Histamine Phosphate Phase 3 51-74-1 65513
20 Neurotransmitter Agents Phase 3
21
Serotonin Phase 3 50-67-9 5202
22 Serotonin Agents Phase 3
23 Serotonin Antagonists Phase 3
24
Dacarbazine Approved, Investigational Phase 2 4342-03-4 5351166
25
Temozolomide Approved, Investigational Phase 2 85622-93-1 5394
26
Dexamethasone Approved, Investigational, Vet_approved Phase 2 50-02-2 5743
27
Hydrocortisone Approved, Vet_approved Phase 2 50-23-7 5754 657311
28
Mifepristone Approved, Investigational Phase 2 84371-65-3 55245
29
Paclitaxel Approved, Vet_approved Phase 2 33069-62-4 36314
30
Trabectedin Approved, Investigational Phase 2 114899-77-3 108150
31
Sunitinib Approved, Investigational Phase 2 341031-54-7, 557795-19-4 5329102
32
nivolumab Approved Phase 2 946414-94-4
33
Saracatinib Investigational Phase 2 379231-04-6
34
Trebananib Investigational Phase 2 894356-79-7
35 Antiemetics Phase 2
36 Anti-Inflammatory Agents Phase 2
37 Antineoplastic Agents, Hormonal Phase 2
38 Autonomic Agents Phase 2
39 BB 1101 Phase 2
40 Cortisol succinate Phase 2
41 Dexamethasone acetate Phase 2 1177-87-3
42 glucocorticoids Phase 2
43 HIV Protease Inhibitors Phase 2
44 Hormone Antagonists Phase 2
45 Hormones Phase 2
46 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2
47 Hydrocortisone 17-butyrate 21-propionate Phase 2
48 Hydrocortisone acetate Phase 2
49 Peripheral Nervous System Agents Phase 2
50
protease inhibitors Phase 2

Interventional clinical trials:

(show all 22)

id 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 Chemotherapy in Treating Patients With Soft Tissue Sarcoma Completed NCT00002641 Phase 3 doxorubicin hydrochloride;ifosfamide;isolated perfusion
3 Surgery With or Without Combination Chemotherapy in Treating Patients With Lung Metastases From Soft Tissue Sarcoma Completed NCT00002764 Phase 3 doxorubicin hydrochloride;ifosfamide
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 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
11 Thalidomide in Treating Patients With Gynecologic Sarcomas Completed NCT00006005 Phase 2 thalidomide
12 Perifosine in Treating Patients With Metastatic or Locally Advanced Soft Tissue Sarcoma Completed NCT00053794 Phase 2 perifosine
13 STI571 in Treating Patients With Recurrent or Refractory Soft Tissue Sarcoma Completed NCT00006357 Phase 1, Phase 2 imatinib mesylate
14 Ecteinascidin 743 in Treating Patients With Advanced Soft Tissue Sarcoma Completed NCT00003939 Phase 2 trabectedin
15 Sunitinib in Treating Patients With Metastatic, Locally Advanced, or Locally Recurrent Sarcomas Completed NCT00474994 Phase 2 sunitinib malate
16 Trebananib in Treating Patients With Persistent or Recurrent Endometrial Cancer Completed NCT01210222 Phase 2
17 BI 2536 in Treating Patients With Recurrent or Metastatic Solid Tumors Completed NCT00526149 Phase 2 BI 2536
18 Phase II Study of Imatinib Mesylate in Patients With Life Threatening Malignant Rare Diseases Completed NCT00154388 Phase 2 Imatinib mesylate
19 A Study of Nivolumab in Selected Uterine Cancer Patients Recruiting NCT03241745 Phase 2 Nivolumab
20 A Phase II of Nivolumab Plus Ipilimumab in Non-resectable Sarcoma and Endometrial Carcinoma Not yet recruiting NCT02982486 Phase 2 Ipilimumab;Nivolumab
21 Collecting Tumor Samples From Patients With Gynecological Tumors Completed NCT00897442
22 Observational Study on Endometrial Stromal Tumors Recruiting NCT02829437

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:

39
Uterus, Smooth Muscle, Lung, Colon, Ovary, Breast, Lymph Node

Publications for Endometrial Stromal Sarcoma

Articles related to Endometrial Stromal Sarcoma:

(show top 50) (show all 489)
id Title Authors Year
1
Magnetic resonance imaging features of endometrial stromal sarcoma: a case description. ( 28275572 )
2017
2
Primary low-grade endometrial stromal sarcoma of the omentum. ( 28831418 )
2017
3
A Rare Collision Tumour of Uterus- Squamous Cell Carcinoma and Endometrial Stromal Sarcoma. ( 28384878 )
2017
4
Clinicopathological characteristics and treatment strategies for patients with low-grade endometrial stromal sarcoma. ( 28403089 )
2017
5
Low-grade and high-grade endometrial stromal sarcoma: A National Cancer Database study. ( 28596015 )
2017
6
Primary extragenital endometrial stromal sarcoma of the lung: first reported case and review of literature. ( 28464838 )
2017
7
Endometrial stromal sarcoma-A retropsective analysis of factors affecting recurrence. ( 28738297 )
2017
8
A recurrent endometrial stromal sarcoma harbors the novel fusion JAZF1-BCORL1. ( 28331900 )
2017
9
YWHAE-rearranged high-grade endometrial stromal sarcoma: Two-center case series and response to chemotherapy. ( 28390819 )
2017
10
An unexpected diagnosis of primary omental endometrial stromal sarcoma in a patient with acute right abdominal pain: A case report and review of literature. ( 28494324 )
2017
11
Low-grade endometrial stromal sarcoma with extensive sex cord differentiation, heterologous elements, and complex atypical hyperplasia: Case report and review of literature. ( 28054022 )
2017
12
Fusion of the genes BRD8 and PHF1 in endometrial stromal sarcoma. ( 28758277 )
2017
13
Androgen Receptor Expression in Endometrial Stromal Sarcoma: Correlation With Clinicopathologic Features. ( 28114189 )
2017
14
YWHAE Rearrangement in a Purely Conventional Low-grade Endometrial Stromal Sarcoma that Transformed Over Time to High-grade Sarcoma: Importance of Molecular Testing. ( 28863072 )
2017
15
Endometrial Stromal Sarcoma of the Uterus: Magnetic Resonance Imaging Findings Including Apparent Diffusion Coefficient Value and Its Correlation With Ki-67 Expression. ( 28906310 )
2017
16
BCOR is a robust diagnostic immunohistochemical marker of genetically diverse high-grade endometrial stromal sarcoma, including tumors exhibiting variant morphology. ( 28621321 )
2017
17
Low-Grade Endometrial Stromal Sarcoma with Intravenous and Intracardiac Extension: A Multidisciplinary Approach. ( 27239357 )
2016
18
Endometrial Stromal Sarcoma Arising in Colorectal Endometriosis. ( 27801759 )
2016
19
Endobronchial metastasised endometrial stromal sarcoma. ( 26643114 )
2016
20
The application of next generation-sequencing-based molecular diagnostics in endometrial stromal sarcoma. ( 26990025 )
2016
21
Novel High-grade Endometrial Stromal Sarcoma: A Morphologic Mimicker of Myxoid Leiomyosarcoma. ( 27631520 )
2016
22
Prolonged response to exemestane following multiple surgical resections and hormonal therapies in a patient with recurrent endometrial stromal sarcoma. ( 26937477 )
2016
23
A case of advanced-stage endometrial stromal sarcoma of the ovary arising from endometriosis. ( 27462602 )
2016
24
Letrozole as second-line hormonal treatment for recurrent low-grade endometrial stromal sarcoma: A case report and review of the literature. ( 27895740 )
2016
25
Endometrial stromal sarcoma: An aggressive uterine malignancy. ( 27761197 )
2016
26
Intravenous low-grade endometrial stromal sarcoma with intracardiac extension: A CASE OF inaccurate tumor location on contrast-enhanced computed tomography. ( 26893856 )
2016
27
An Unusual Case of YWHAE-NUTM2A/B Endometrial Stromal Sarcoma With Confinement to the Endometrium and Lack of High-Grade Morphology. ( 27171540 )
2016
28
Uterine leiomyosarcoma and endometrial stromal sarcoma have unique miRNA signatures. ( 26768834 )
2016
29
Immunohistochemical Characterization of Histone Deacetylase as a Potential Prognostic Marker and Therapeutic Target in Endometrial Stromal Sarcoma. ( 27127168 )
2016
30
Uterine sarcoma Part II-Uterine endometrial stromal sarcoma: The TAG systematic review. ( 27590366 )
2016
31
Fertility-sparing surgery for patients with low-grade endometrial stromal sarcoma. ( 27736798 )
2016
32
Cytogenetic and Molecular Profile of Endometrial Stromal Sarcoma. ( 27219024 )
2016
33
Ultrasonographic findings of low-grade endometrial stromal sarcoma of the uterus with a focus on cystic degeneration. ( 26537303 )
2015
34
Influence of different treatment modalities on survival of patients with low-grade endometrial stromal sarcoma: A retrospective cohort study. ( 26449652 )
2015
35
Long-term survival in low-grade endometrial stromal sarcoma with childbirth and multidisciplinary treatment: a case report. ( 26481040 )
2015
36
Endometrial stromal sarcoma with endometrioid adenocarcinoma of the uterus: a case report. ( 26191224 )
2015
37
Endometrial Stromal Sarcoma with Intravenous and Cardiac Extension. ( 26375498 )
2015
38
Endometrial stromal sarcoma presenting as large bleeding left upper quadrant mass. ( 26605285 )
2015
39
Genomic landscape of endometrial stromal sarcoma of uterus. ( 26429873 )
2015
40
Osteosarcoma arising from metastatic low grade endometrial stromal sarcoma. ( 25474520 )
2015
41
Complete remission achieved by oophorectomy for recurrent endometrial stromal sarcoma after laparoscopic morcellation. ( 26076082 )
2015
42
Prognostic factors, treatment and outcome in a Turkish population with endometrial stromal sarcoma. ( 25735377 )
2015
43
Delayed osseous metastasis from low-grade endometrial stromal sarcoma: Uncommon occurrence deserving recognition. ( 26171849 )
2015
44
Two-stage resection of a disseminated mixed endometrial stromal sarcoma and smooth muscle tumor with intravascular and intracardiac extension. ( 26701002 )
2015
45
Long-Term Outcome of Aromatase Inhibitor Therapy With Letrozole in Patients With Advanced Low-Grade Endometrial Stromal Sarcoma. ( 26495759 )
2015
46
Cyclin D1 Does Not Distinguish YWHAE-NUTM2 High-grade Endometrial Stromal Sarcoma From Undifferentiated Endometrial Carcinoma. ( 25871469 )
2015
47
Heart and Lung Metastases From Endometrial Stromal Sarcoma in a Forty-Two-Year-Old Woman. ( 26436070 )
2015
48
Low-grade Endometrial Stromal Sarcoma Primarily Arising in the Vagina: A Case Report. ( 26592071 )
2015
49
Fertility sparing surgery in young women affected by endometrial stromal sarcoma: an oncologic dilemma or a reliable option? review of literature starting from a peculiar case. ( 25565863 )
2015
50
Recurrent cerebral infarction synchronous with menorrhagia caused by endometrial stromal sarcoma. ( 26432578 )
2015

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:

id Super pathways Score Top Affiliating Genes
1 11.81 CCND1 CYP19A1 ESR1 TP53
2 11.44 ACTC1 DES VIM
3 11.36 CCND1 ESR1 YWHAE
4 11.06 ESR1 TP53 YWHAE
5 10.66 BCOR ESR1 YWHAE
6 10.3 CYP19A1 INHA PGR
7 9.76 CYP19A1 ESR1

GO Terms for Endometrial Stromal Sarcoma

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

id Name GO ID Score Top Affiliating Genes
1 negative regulation of transcription from RNA polymerase II promoter GO:0000122 9.63 BCOR CCND1 ESR1 JAZF1 SUZ12 TP53
2 replicative senescence GO:0090399 9.32 MME TP53
3 mitotic G1 DNA damage checkpoint GO:0031571 9.26 CCND1 TP53
4 positive regulation of phospholipase C activity GO:0010863 8.96 ESR1 KIT
5 muscle filament sliding GO:0030049 8.8 ACTC1 DES VIM

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

id Name GO ID Score Top Affiliating Genes
1 enzyme binding GO:0019899 9.35 CCND1 ESR1 PGR TP53 YWHAE
2 histone deacetylase binding GO:0042826 8.92 BCOR CCND1 TP53 YWHAE

Sources for Endometrial Stromal Sarcoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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