MCID: END031
MIFTS: 53

Endometrial Stromal Sarcoma

Categories: Rare diseases, Cancer diseases, Reproductive diseases

Aliases & Classifications for Endometrial Stromal Sarcoma

MalaCards integrated aliases for Endometrial Stromal Sarcoma:

Name: Endometrial Stromal Sarcoma 12 72 49 55 14 69
Ess 12 49
Endometrial Stromal Sarcoma, High Grade 12
Undifferentiated Endometrial Sarcoma 12
Stromal Sarcoma of the Corpus Uteri 55
Undifferentiated Stromal Sarcoma 69
Sarcoma, Endometrial Stromal 41
Sarcoma Endometrial Stromal 51

Classifications:



External Ids:

Disease Ontology 12 DOID:4226
MeSH 41 D018203
NCIt 46 C8973
SNOMED-CT 64 699356008 70555003
Orphanet 55 ORPHA213711
MESH via Orphanet 42 D018203
UMLS via Orphanet 70 C0206630
ICD10 via Orphanet 33 C54.1

Summaries for Endometrial Stromal Sarcoma

NIH Rare Diseases : 49 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 endometrial stromal nodule and leiomyosarcoma. An important gene associated with Endometrial Stromal Sarcoma is SUZ12 (SUZ12 Polycomb Repressive Complex 2 Subunit), and among its related pathways/superpathways are Gastric cancer and Integrated Breast Cancer Pathway. 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 202)
# Related Disease Score Top Affiliating Genes
1 endometrial stromal nodule 31.7 BCOR CYP19A1 JAZF1 MEAF6 MME NUTM2A
2 leiomyosarcoma 31.0 ACTC1 DES KIT PGR TP53 VIM
3 intravenous leiomyomatosis 31.0 DES ESR1 MME
4 leiomyoma 31.0 ACTC1 CYP19A1 DES ESR1 MME PGR
5 uterine carcinosarcoma 30.9 KIT PGR TP53
6 myoma 30.9 CYP19A1 ESR1 PGR
7 lymphangioleiomyomatosis 30.8 DES ESR1 PGR
8 uterine sarcoma 30.8 CYP19A1 DES ESR1 KIT MME
9 smooth muscle tumor 30.8 ACTC1 DES ESR1 KIT MME PGR
10 endometriosis of ovary 30.7 CYP19A1 ESR1 PGR
11 ossifying fibromyxoid tumor 30.7 ACTC1 DES VIM
12 hemangiopericytoma, malignant 30.7 ACTC1 DES VIM
13 endometrial adenocarcinoma 30.5 ESR1 PGR TP53
14 adenofibroma 30.5 ACTC1 INHA MME VIM
15 myxoid leiomyosarcoma 30.4 ACTC1 BCOR PGR YWHAE ZC3H7B
16 uterine corpus endometrial stromal sarcoma 12.2
17 cervical endometrial stromal sarcoma 12.2
18 vaginal endometrial stromal sarcoma 12.2
19 empty sella syndrome 11.6
20 sarcoma 11.2
21 euthyroid sick syndrome 10.9
22 skin glomus tumor 10.7 DES VIM
23 vulvar syringoma 10.7 ESR1 PGR
24 infantile digital fibromatosis 10.7 DES VIM
25 lung leiomyoma 10.7 ESR1 PGR
26 lymphangiectasis 10.7 ACTC1 VIM
27 glassy cell carcinoma of the cervix 10.7 ESR1 PGR
28 lymphangiomatosis 10.7 DES PGR VIM
29 breast myofibroblastoma 10.7 ESR1 MME
30 epulis 10.7 DES PGR VIM
31 progesterone-receptor positive breast cancer 10.7 CYP19A1 ESR1 PGR
32 vulvar benign neoplasm 10.7 ESR1 PGR
33 endometrium carcinoma in situ 10.7 PGR TP53
34 trigonitis 10.7 ESR1 PGR
35 gender identity disorder 10.7 CYP19A1 ESR1 PGR
36 vulvar leiomyoma 10.7 ESR1 PGR
37 estrogen excess 10.7 CYP19A1 ESR1 PGR
38 mammographic density 10.7 CYP19A1 ESR1 PGR
39 endometrial mucinous adenocarcinoma 10.7 ESR1 PGR VIM
40 calcifying aponeurotic fibroma 10.7 ACTC1 PGR
41 angiolipoma 10.7 ACTC1 DES VIM
42 cutaneous leiomyosarcoma 10.7 ACTC1 DES VIM
43 spindle cell lipoma 10.7 ACTC1 DES VIM
44 parachordoma 10.7 ACTC1 DES VIM
45 congenital epulis 10.7 DES PGR VIM
46 fibroblastic rheumatism 10.7 ACTC1 DES
47 epithelioid leiomyosarcoma 10.7 ACTC1 DES VIM
48 syringoma 10.7 DES ESR1 VIM
49 syringocystadenoma papilliferum 10.7 ACTC1 DES VIM
50 x-ray sensitivity 10.6 PHF1 TP53

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:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.18 DES ESR1 TP53 ACTC1 INHA BCOR
2 cellular MP:0005384 10.15 PGR DES SUZ12 ESR1 ACTC1 BCOR
3 homeostasis/metabolism MP:0005376 10.1 CYP19A1 PGR DES ESR1 ACTC1 INHA
4 immune system MP:0005387 10 PGR SUZ12 ESR1 INHA BCOR CCND1
5 muscle MP:0005369 9.81 DES ESR1 TP53 ACTC1 KIT CYP19A1
6 neoplasm MP:0002006 9.5 PGR SUZ12 ESR1 INHA CCND1 KIT
7 reproductive system MP:0005389 9.28 CYP19A1 PGR ESR1 INHA CCND1 KIT

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)
# 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
Mechlorethamine Approved, Investigational Phase 3 51-75-2 4033
4
Lenograstim Approved, Investigational Phase 3 135968-09-1
5
Histamine Approved, Investigational Phase 3 51-45-6, 75614-87-8 774
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 Isophosphamide mustard Phase 3 0
10 Gastrointestinal Agents Phase 3,Phase 2
11 Topoisomerase Inhibitors Phase 3,Phase 2
12 Anti-Bacterial Agents Phase 3,Phase 2
13 Antibiotics, Antitubercular Phase 3,Phase 2
14
Histamine Phosphate Phase 3 51-74-1 65513
15
Serotonin Phase 3 50-67-9 5202
16 Serotonin Agents Phase 3
17 Serotonin Antagonists Phase 3
18 Neurotransmitter Agents Phase 3
19 Histamine Antagonists Phase 3
20 Histamine H1 Antagonists Phase 3
21 Dermatologic Agents Phase 3
22 Anti-Allergic Agents Phase 3
23 Antipruritics 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
Mifepristone Approved, Investigational Phase 2 84371-65-3 55245
28
Paclitaxel Approved, Vet_approved Phase 2 33069-62-4 36314
29
Hydrocortisone Approved, Vet_approved Phase 2 50-23-7 657311 5754
30
Sunitinib Approved, Investigational Phase 2 341031-54-7, 557795-19-4 5329102
31
Trabectedin Approved, Investigational Phase 2 114899-77-3 108150
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 Dexamethasone acetate Phase 2 1177-87-3
36 glucocorticoids Phase 2
37
protease inhibitors Phase 2
38 Albumin-Bound Paclitaxel Phase 2
39 Contraceptive Agents Phase 2
40 Contraceptives, Oral Phase 2
41 Contraceptives, Postcoital Phase 2
42 Cortisol succinate Phase 2
43 HIV Protease Inhibitors Phase 2
44 Hormone Antagonists Phase 2
45 Angiogenesis Inhibitors Phase 2
46 Hormones Phase 2
47 Angiogenesis Modulating Agents Phase 2
48 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 2
49 Peripheral Nervous System Agents Phase 2
50 Hydrocortisone 17-butyrate 21-propionate Phase 2

Interventional clinical trials:

(show all 21)

# 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 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

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:

38
Uterus, Smooth Muscle, Lung, Colon, Ovary, Heart, Lymph Node

Publications for Endometrial Stromal Sarcoma

Articles related to Endometrial Stromal Sarcoma:

(show top 50) (show all 496)
# Title Authors Year
1
A remarkable response to pazopanib, despite recurrent liver toxicity, in a patient with a high grade endometrial stromal sarcoma, a case report. ( 29357824 )
2018
2
Immunohistochemical Expression of Different Subtypes of Cytokeratins by Endometrial Stromal Sarcoma. ( 29406332 )
2018
3
Clinicopathological characteristics and treatment strategies for patients with low-grade endometrial stromal sarcoma. ( 28403089 )
2017
4
Endometrial stromal sarcoma-A retropsective analysis of factors affecting recurrence. ( 28738297 )
2017
5
Androgen Receptor Expression in Endometrial Stromal Sarcoma: Correlation With Clinicopathologic Features. ( 28114189 )
2017
6
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
7
Primary low-grade endometrial stromal sarcoma of the omentum. ( 28831418 )
2017
8
YWHAE-rearranged high-grade endometrial stromal sarcoma: Two-center case series and response to chemotherapy. ( 28390819 )
2017
9
BCOR is a robust diagnostic immunohistochemical marker of genetically diverse high-grade endometrial stromal sarcoma, including tumors exhibiting variant morphology. ( 28621321 )
2017
10
Endometrial stromal sarcoma in combination with mixed type endometrial carcinomas: A case report and literature review. ( 29245257 )
2017
11
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
12
Oncogenic HrasG12V expression plus knockdown of Cdkn2a using ecotropic lentiviral vectors induces high-grade endometrial stromal sarcoma. ( 28982163 )
2017
13
Cardiac metastatic endometrial stromal sarcoma 17 years after hysterectomy. ( 28967156 )
2017
14
Fusion of the genes BRD8 and PHF1 in endometrial stromal sarcoma. ( 28758277 )
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
Extrauterine, Extragenital Endometrial Stromal Sarcoma in the Absence of Endometriosis: A Rare Entity. ( 29210799 )
2017
17
Magnetic resonance imaging features of endometrial stromal sarcoma: a case description. ( 28275572 )
2017
18
Primary extragenital endometrial stromal sarcoma of the lung: first reported case and review of literature. ( 28464838 )
2017
19
Low-grade and high-grade endometrial stromal sarcoma: A National Cancer Database study. ( 28596015 )
2017
20
Primary low-grade endometrial stromal sarcoma arising in the vagina: report of an unusual case and literature review. ( 29218216 )
2017
21
A Rare Collision Tumour of Uterus- Squamous Cell Carcinoma and Endometrial Stromal Sarcoma. ( 28384878 )
2017
22
A recurrent endometrial stromal sarcoma harbors the novel fusion JAZF1-BCORL1. ( 28331900 )
2017
23
Low-grade endometrial stromal sarcoma with extensive sex cord differentiation, heterologous elements, and complex atypical hyperplasia: Case report and review of literature. ( 28054022 )
2017
24
Novel High-grade Endometrial Stromal Sarcoma: A Morphologic Mimicker of Myxoid Leiomyosarcoma. ( 27631520 )
2016
25
Endobronchial metastasised endometrial stromal sarcoma. ( 26643114 )
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
Endometrial Stromal Sarcoma Arising in Colorectal Endometriosis. ( 27801759 )
2016
28
Prolonged response to exemestane following multiple surgical resections and hormonal therapies in a patient with recurrent endometrial stromal sarcoma. ( 26937477 )
2016
29
The application of next generation-sequencing-based molecular diagnostics in endometrial stromal sarcoma. ( 26990025 )
2016
30
Fertility-sparing surgery for patients with low-grade endometrial stromal sarcoma. ( 27736798 )
2016
31
Low-Grade Endometrial Stromal Sarcoma with Intravenous and Intracardiac Extension: A Multidisciplinary Approach. ( 27239357 )
2016
32
An Unusual Case of YWHAE-NUTM2A/B Endometrial Stromal Sarcoma With Confinement to the Endometrium and Lack of High-Grade Morphology. ( 27171540 )
2016
33
Uterine sarcoma Part II-Uterine endometrial stromal sarcoma: The TAG systematic review. ( 27590366 )
2016
34
Cytogenetic and Molecular Profile of Endometrial Stromal Sarcoma. ( 27219024 )
2016
35
Uterine leiomyosarcoma and endometrial stromal sarcoma have unique miRNA signatures. ( 26768834 )
2016
36
Letrozole as second-line hormonal treatment for recurrent low-grade endometrial stromal sarcoma: A case report and review of the literature. ( 27895740 )
2016
37
Endometrial stromal sarcoma: An aggressive uterine malignancy. ( 27761197 )
2016
38
Immunohistochemical Characterization of Histone Deacetylase as a Potential Prognostic Marker and Therapeutic Target in Endometrial Stromal Sarcoma. ( 27127168 )
2016
39
A case of advanced-stage endometrial stromal sarcoma of the ovary arising from endometriosis. ( 27462602 )
2016
40
Endometrial stromal sarcoma presenting as large bleeding left upper quadrant mass. ( 26605285 )
2015
41
Long-Term Outcome of Aromatase Inhibitor Therapy With Letrozole in Patients With Advanced Low-Grade Endometrial Stromal Sarcoma. ( 26495759 )
2015
42
Osteosarcoma arising from metastatic low grade endometrial stromal sarcoma. ( 25474520 )
2015
43
Heart and Lung Metastases From Endometrial Stromal Sarcoma in a Forty-Two-Year-Old Woman. ( 26436070 )
2015
44
Delayed osseous metastasis from low-grade endometrial stromal sarcoma: Uncommon occurrence deserving recognition. ( 26171849 )
2015
45
Immunohistochemical panel to differentiate endometrial stromal sarcoma, uterine leiomyosarcoma and leiomyoma: something old and something new. ( 25991737 )
2015
46
Fertility-sparing treatment of low-grade endometrial stromal sarcoma. ( 26131171 )
2015
47
Ultrasonographic findings of low-grade endometrial stromal sarcoma of the uterus with a focus on cystic degeneration. ( 26537303 )
2015
48
Effects of Morcellation of Uterine Smooth Muscle Tumor of Uncertain Malignant Potential and Endometrial Stromal Sarcoma: Case Series and Recommendations for Clinical Practice. ( 25596464 )
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

GO Terms for Endometrial Stromal Sarcoma

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

# Name GO ID Score Top Affiliating Genes
1 negative regulation of transcription by RNA polymerase II 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
6 transcription, DNA-templated GO:0006351 10.02 BCOR CCND1 ESR1 JAZF1 MEAF6 PGR

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

# 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
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 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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