MCID: EPT012
MIFTS: 48

Epithelioid Sarcoma

Categories: Cancer diseases, Rare diseases

Aliases & Classifications for Epithelioid Sarcoma

MalaCards integrated aliases for Epithelioid Sarcoma:

Name: Epithelioid Sarcoma 12 52 58 15
Epithelioid Cell Sarcoma 12
Sarcoma, Epithelioid 71
Sarcoma Epithelioid 54

Characteristics:

Orphanet epidemiological data:

58
epithelioid sarcoma
Inheritance: Not applicable; Prevalence: <1/1000000 (Europe); Age of onset: Adolescent,Adult,Childhood; Age of death: young Adult;

Classifications:



External Ids:

Disease Ontology 12 DOID:6193
NCIt 49 C3714
SNOMED-CT 67 59238007
ICD10 via Orphanet 33 C49.9
UMLS via Orphanet 72 C0205944
Orphanet 58 ORPHA293202
UMLS 71 C0205944

Summaries for Epithelioid Sarcoma

NIH Rare Diseases : 52 Epithelioid sarcoma is a rare cancer that most often occurs in the soft tissue of the fingers, hands and forearms of young adults. It may also be found in the legs, trunk, head or neck regions. It is rare in young children and adults, and it occurs more frequently in men. Epithelioid sarcoma begins as a painless, firm growth or bump that may be accompanied by an open wound (ulceration ) in the skin covering the growth. It is considered an aggressive cancer because it has a high chance of regrowing after treatment (a recurrence ), or spreading to surrounding tissues or more distant parts of the body (a metastasis ). Epithelioid sarcoma is first treated with surgery to remove all the cancer cells (wide local excision ). Amputation of part of the affected limb may be needed in severe cases. Radiation therapy or chemotherapy may also be used to destroy any cancer cells not removed during surgery.

MalaCards based summary : Epithelioid Sarcoma, also known as epithelioid cell sarcoma, is related to central epithelioid sarcoma and vulvar proximal-type epithelioid sarcoma. An important gene associated with Epithelioid Sarcoma is SMARCB1 (SWI/SNF Related, Matrix Associated, Actin Dependent Regulator Of Chromatin, Subfamily B, Member 1), and among its related pathways/superpathways are Cytoskeletal Signaling and Cell adhesion molecules (CAMs). The drugs Mechlorethamine and Ifosfamide have been mentioned in the context of this disorder. Affiliated tissues include breast, skin and lung, and related phenotypes are Reduced mammosphere formation and cardiovascular system

Disease Ontology : 12 A connective tissue cancer that is characterized by the presence of epithelioid cells forming nodular patterns and has material basis in mesenchymal tissue.

Wikipedia : 74 Epithelioid sarcoma is a rare soft tissue sarcoma arising from mesenchymal tissue and characterized by... more...

Related Diseases for Epithelioid Sarcoma

Diseases in the Epithelioid Sarcoma family:

Central Epithelioid Sarcoma Adult Epithelioid Sarcoma

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

(show top 50) (show all 501)
# Related Disease Score Top Affiliating Genes
1 central epithelioid sarcoma 33.7 VIM SYP SMARCB1 KRT7 CD34
2 vulvar proximal-type epithelioid sarcoma 33.7 VIM SYP SMARCB1 KRT7 CD34
3 epithelioid cell synovial sarcoma 32.5 SYP KRT7 CD34 CALB2
4 spindle cell sarcoma 31.9 VIM MUC1 KRT7 CD34
5 rhabdoid tumor predisposition syndrome 1 31.4 VIM SYP SMARCB1 MUC1
6 rhabdoid cancer 31.1 SYP SMARCB1 PBRM1
7 pneumothorax 30.9 VIM MUC1 CALB2
8 villonodular synovitis 30.8 VIM CD68
9 meningioma, radiation-induced 30.7 VIM SMARCB1 MUC1
10 secretory meningioma 30.7 VIM MUC1 KRT7
11 myoepithelioma 30.7 VIM SMARCB1 MUC1
12 vulvar sarcoma 30.6 SMARCB1 CD34
13 atypical teratoid rhabdoid tumor 30.6 VIM SYP SMARCB1 MUC1
14 leiomyosarcoma 30.6 VIM PECAM1 MUC1
15 myoepithelial carcinoma 30.5 VIM SMARCB1 MUC1 KRT7
16 pleomorphic liposarcoma 30.5 VIM MUC1 KRT8
17 rhabdoid meningioma 30.5 VIM SYP SMARCB1
18 sweat gland cancer 30.4 SYP KRT8 KRT7
19 pseudosarcomatous fibromatosis 30.3 VIM CD68 CD34
20 malignant fibroxanthoma 30.3 VIM MUC1 CD68 CD34
21 cutaneous fibrous histiocytoma 30.2 VIM CD68 CD34
22 fibrous histiocytoma 30.2 VIM PECAM1 CD68 CD34
23 histiocytoma 30.2 VIM PECAM1 CD68 CD34
24 alveolar soft part sarcoma 30.2 VIM SYP EGFR
25 epithelioid malignant peripheral nerve sheath tumor 30.2 SMARCB1 MUC1 CD34
26 biphasic synovial sarcoma 30.1 VIM MUC1 CDH1 CALB2
27 extraosseous chondrosarcoma 30.1 SYP SMARCB1 CD34
28 benign mesothelioma 30.1 PECAM1 KRT7 CD34 CALB2
29 chondrosarcoma, extraskeletal myxoid 30.0 SYP SMARCB1 CD34
30 in situ carcinoma 30.0 MUC1 KRT8 EGFR CDH1
31 myxoid chondrosarcoma 30.0 SYP SMARCB1 MUC1 CD68
32 vulvar disease 30.0 KRT7 EGFR CD34
33 malignant pleural mesothelioma 30.0 MUC1 EGFR CALB2
34 mesenchymal cell neoplasm 29.9 PECAM1 CD68 CD34
35 chordoma 29.8 VIM SMARCB1 MUC1 KRT7 EGFR CDH1
36 sarcoma, synovial 29.8 VIM SMARCB1 MUC1 KRT8 KRT7 CDH1
37 connective tissue benign neoplasm 29.8 PECAM1 CD68 CD34 CALB2
38 exophthalmos 29.8 SYP CD68 CD34
39 fibroma 29.8 VIM PECAM1 KRT7 CD68 CD34 CALB2
40 neurilemmoma 29.7 VIM SMARCB1 MUC1 CD34 CALB2
41 monophasic synovial sarcoma 29.7 VIM MUC1 KRT7 CD34 CALB2
42 liposarcoma 29.6 VIM CD68 CD34 CALB2
43 malignant peripheral nerve sheath tumor 29.6 VIM SMARCB1 MUC1 KRT7 EGFR CD34
44 extraosseous osteosarcoma 29.4 CD68 CD34
45 vascular cancer 29.2 SYP PECAM1 KRT7 CD68 CD34
46 angiosarcoma 29.2 VIM PECAM1 MUC1 KRT8 KRT7 CD68
47 spindle cell carcinoma 29.0 VIM SYP MUC1 KRT8 KRT7 CDH1
48 glioblastoma multiforme 29.0 SYP PECAM1 MIR671 EGFR CDH5 CDH1
49 meningioma, familial 28.7 SYP SMARCB1 PECAM1 EGFR CDH1 CD68
50 lung cancer susceptibility 3 28.6 PECAM1 MUC1 MIR671 KRT8 KRT7 EGFR

Graphical network of the top 20 diseases related to Epithelioid Sarcoma:



Diseases related to Epithelioid Sarcoma

Symptoms & Phenotypes for Epithelioid Sarcoma

GenomeRNAi Phenotypes related to Epithelioid Sarcoma according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Reduced mammosphere formation GR00396-S 9.17 CD34 CDH1 CDH5 EGFR KRT7 SMARCB1

MGI Mouse Phenotypes related to Epithelioid Sarcoma:

45
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.65 CDH1 CDH5 EGFR KRT1 KRT8 PBRM1
2 immune system MP:0005387 9.44 CD109 CD34 CD68 CDH1 CDH5 EGFR

Drugs & Therapeutics for Epithelioid Sarcoma

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

(show top 50) (show all 52)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Mechlorethamine Approved, Investigational Phase 3 51-75-2 4033
2
Ifosfamide Approved Phase 3 3778-73-2 3690
3
Lenograstim Approved, Investigational Phase 3 135968-09-1
4
Epirubicin Approved Phase 3 56420-45-2 41867
5
Dactinomycin Approved, Investigational Phase 3 50-76-0 2019 457193
6
Cyclophosphamide Approved, Investigational Phase 3 50-18-0, 6055-19-2 2907
7
Vincristine Approved, Investigational Phase 3 2068-78-2, 57-22-7 5978
8
Carboplatin Approved Phase 3 41575-94-4 10339178 498142 38904
9
Etoposide Approved Phase 3 33419-42-0 36462
10
Doxorubicin Approved, Investigational Phase 2, Phase 3 23214-92-8 31703
11
Trabectedin Approved, Investigational Phase 2, Phase 3 114899-77-3 108150
12
Histamine Approved, Investigational Phase 3 51-45-6 774
13
Cyproheptadine Approved Phase 3 129-03-3 2913
14 Immunologic Factors Phase 3
15
Isophosphamide mustard Phase 3 0
16 Immunosuppressive Agents Phase 3
17 Anti-Infective Agents Phase 3
18 Antirheumatic Agents Phase 3
19 Etoposide phosphate Phase 3
20 Antimitotic Agents Phase 3
21 Anti-Bacterial Agents Phase 2, Phase 3
22 Antibiotics, Antitubercular Phase 2, Phase 3
23 Topoisomerase Inhibitors Phase 2, Phase 3
24
Liposomal doxorubicin Phase 2, Phase 3 31703
25 Alkylating Agents Phase 2, Phase 3
26 Gastrointestinal Agents Phase 3
27 Neurotransmitter Agents Phase 3
28 Dermatologic Agents Phase 3
29 Antipruritics Phase 3
30 Histamine H1 Antagonists Phase 3
31 Serotonin Agents Phase 3
32 Histamine Antagonists Phase 3
33
Histamine Phosphate Phase 3 51-74-1 65513
34 Serotonin Antagonists Phase 3
35 Anti-Allergic Agents Phase 3
36
Serotonin Investigational, Nutraceutical Phase 3 50-67-9 5202
37
Temozolomide Approved, Investigational Phase 2 85622-93-1 5394
38
Dasatinib Approved, Investigational Phase 2 302962-49-8 3062316
39
Sunitinib Approved, Investigational Phase 2 341031-54-7, 557795-19-4 5329102
40
Durvalumab Approved, Investigational Phase 2 1428935-60-7
41 Protein Kinase Inhibitors Phase 2
42 Imatinib Mesylate Phase 1, Phase 2 220127-57-1 123596
43 Angiogenesis Inhibitors Phase 2
44 lysine Phase 2
45 Antibodies, Monoclonal Phase 2
46 Antineoplastic Agents, Immunological Phase 2
47 Immunoglobulins Phase 2
48 Antibodies Phase 2
49 Orange Approved
50
Mesna Approved, Investigational 3375-50-6 598

Interventional clinical trials:

(show all 30)
# Name Status NCT ID Phase Drugs
1 Localized Non-Rhabdomyosarcoma Soft Tissue Sarcomas Unknown status NCT00334854 Phase 3 doxorubicin hydrochloride;ifosfamide
2 Randomised Trial of Post-Operative Radiotherapy Given to Adult Patients With Extremity Soft Tissue Sarcoma [VORTEX] Completed NCT00423618 Phase 3
3 Randomised Trial Of Single Agent Doxorubicin Versus Doxorubicin Plus Ifosfamide In The First Line Treatment Of Advanced Or Metastatic Soft Tissue Sarcoma Completed NCT00061984 Phase 3 doxorubicin hydrochloride;ifosfamide
4 A Phase III Randomized Study of Preoperative Radiation Plus Surgery Versus Surgery Alone for Patients With Retroperitoneal Sarcomas (RPS) Completed NCT00091351 Phase 3
5 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
6 MMT 95 Study For Rhabdomyosarcoma and Other Malignant Soft Tissue Tumors of Childhood Completed NCT00002898 Phase 3 carboplatin;cyclophosphamide;epirubicin hydrochloride;etoposide;ifosfamide;vincristine sulfate
7 A Phase 1b/3 Global, Randomized, Double-blind, Placebo-Controlled Trial of Tazemetostat in Combination With Doxorubicin as Frontline Therapy for Advanced Epithelioid Sarcoma Recruiting NCT04204941 Phase 3 Tazemetostat;Placebo;Doxorubicin HCl
8 TRUSTS: A Phase IIB/III Multicenter Study Comparing the Efficacy of TRabectedin Administered as a 3-Hour or 24-Hour Infusion to Doxorubicin in Patients With Advanced or Metastatic Untreated Soft Tissue Sarcoma Terminated NCT01189253 Phase 2, Phase 3 doxorubicin hydrochloride;trabectedin
9 Prevention of Cancer/Treatment-Related Weight Loss in Children at High Nutritional Risk Terminated NCT01132547 Phase 3 cyproheptadine hydrochloride
10 Phase II Evaluation of Temodal (Temozolomide, Schering) in Previously Treated Advanced Sarcomas Unknown status NCT00003718 Phase 2 temozolomide
11 Randomized Phase II Study of Brostallicin (PNU-166196A) Versus Doxorubicin as First Line Chemotherapy in Patients With Advanced or Metastatic Soft Tissue Sarcoma Unknown status NCT00410462 Phase 2 brostallicin;doxorubicin hydrochloride
12 Phase II Study on ET-743 in Advanced Soft Tissue Sarcomas of the Adult Completed NCT00003939 Phase 2 trabectedin
13 A Phase II Trial of Dasatinib in Advanced Sarcomas Completed NCT00464620 Phase 2 Dasatinib
14 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
15 Dose Finding and Phase II Study of STI 571 in Advanced Soft Tissue Sarcoma Completed NCT00006357 Phase 1, Phase 2 imatinib mesylate
16 A Multicenter Phase II Study of Continuous Dosing of Sunitinib (Sutent®, SU11248) in Non-GIST Sarcomas Completed NCT00474994 Phase 2 sunitinib malate
17 MMT 98 Study For Metastatic Disease Rhabdomyosarcoma And Other Malignant Soft Tissue Sarcoma Of Childhood Completed NCT00025441 Phase 2 carboplatin;cyclophosphamide;doxorubicin hydrochloride;epirubicin hydrochloride;etoposide;ifosfamide;vincristine sulfate
18 A Phase II, Multicenter Study of the EZH2 Inhibitor Tazemetostat in Adult Subjects With INI1-Negative Tumors or Relapsed/Refractory Synovial Sarcoma Recruiting NCT02601950 Phase 2 Tazemetostat
19 A Phase II Trial of Pazopanib and Durvalumab for Metastatic Soft Tissue Sarcoma Recruiting NCT03798106 Phase 2 Durvalumab, pazopanib
20 Phase II Study of Preoperative Intensity-Modulated Radiation Therapy for Soft-Tissue Sarcomas Terminated NCT00740597 Phase 2
21 Phase I Study To Determine The Safety Of Caelyx (Doxorubin HCI, Pegylated Liposomal) In Combination With Ifosfamide In Previously Untreated Adult Patients With Advanced And/Or Metastatic Soft Tissues Sarcomas Completed NCT00030784 Phase 1 ifosfamide;pegylated liposomal doxorubicin hydrochloride
22 A Phase 1 Study of the EZH2 Inhibitor Tazemetostat in Pediatric Subjects With Relapsed or Refractory INI1-Negative Tumors or Synovial Sarcoma Recruiting NCT02601937 Phase 1 Tazemetostat
23 VORTEX-BIOBANK: Prospective Sample Collection for the VORTEX Randomised Radiotherapy Trial in Patients With Extremity Soft Tissue Sarcoma [VORTEX BIOBANK] Unknown status NCT00900211
24 Epithelioid Sarcoma. An Observational Study Recruiting NCT03099681
25 MYometrial Lesion UltrasouNd And mRi Recruiting NCT04123158
26 Epithelioid Sarcoma Natural History Study Active, not recruiting NCT03837678
27 An Evaluation of PET/CT Imaging as a Predictor of Disease Free Survival Following Neo-Adjuvant Chemotherapy for Soft Tissue Sarcoma Active, not recruiting NCT00346125 doxorubicin hydrochloride;ifosfamide;pegylated liposomal doxorubicin hydrochloride
28 Multimodal Immune Characterization of RAre Soft Tissue Sarcoma - MIRAS Project From SARRA (SARcome RAre) Project of the French Sarcoma Group Not yet recruiting NCT03967834
29 Tazemetostat Expanded Access Program for Adults With Solid Tumors Temporarily not available NCT03874455 Tazemetostat
30 Prevention of Mucositis in Children With AES-14 (IND#36978), a Glutamine Based Oral Care Regimen, for Patients Diagnosed With Solid Tumors: A Randomized Placebo-Controlled Clinical Study Withdrawn NCT00334984 glutamine

Search NIH Clinical Center for Epithelioid Sarcoma

Genetic Tests for Epithelioid Sarcoma

Anatomical Context for Epithelioid Sarcoma

MalaCards organs/tissues related to Epithelioid Sarcoma:

40
Breast, Skin, Lung, Bone, Ovary, Heart, Liver

Publications for Epithelioid Sarcoma

Articles related to Epithelioid Sarcoma:

(show top 50) (show all 840)
# Title Authors PMID Year
1
[Epithelioid sarcoma: morphologic variants and differential diagnosis]. 54 61
19997734 2010
2
Epithelioid sarcoma of the parotid gland of a child. 54 61
19335024 2009
3
Primary epithelioid sarcoma of bone: report of a unique case, with immunohistochemical and fluorescent in situ hybridization confirmation of INI1 deletion. 54 61
19342946 2009
4
INI1 and GLUT-1 expression in epithelioid sarcoma and its cutaneous neoplastic and nonneoplastic mimics. 54 61
19318800 2009
5
Loss of INI1 expression is characteristic of both conventional and proximal-type epithelioid sarcoma. 54 61
19033866 2009
6
Epithelioid sarcoma: a case report with ultrastructural confirmation of myofibroblastic differentiation based on fibronexus junctions. 54 61
19274582 2009
7
Epithelioid sarcoma: a clinicopathologic and immunohistochemical analysis of 106 cases from the French sarcoma group. 54 61
19141382 2009
8
Epithelioid sarcoma of Enzinger. 54 61
16778474 2006
9
Evaluation of perineurial differentiation in epithelioid sarcoma. 54 61
16324194 2005
10
CA125 expression in epithelioid sarcoma. 54 61
15078911 2004
11
Epithelioid sarcoma: new insights based on an extended immunohistochemical analysis. 54 61
12946229 2003
12
Cytokeratin 7 and 20 expression in epithelioid sarcoma. 54 61
12680954 2003
13
Epithelioid sarcomas with elevated serum CA125: report of two cases. 54 61
12672792 2003
14
Epithelioid sarcoma: an immunohistochemical analysis evaluating the utility of cytokeratin 5/6 in distinguishing superficial epithelioid sarcoma from spindled squamous cell carcinoma. 54 61
12641789 2003
15
Keratin 1 expression in endothelia and mesenchymal tumors: an immunohistochemical analysis of normal and neoplastic tissues. 54 61
11521233 2001
16
Spindle cell variant of epithelioid sarcoma: an easily misdiagnosed tumour. 54 61
11309041 2001
17
Growth inhibitory effects of paclitaxel on human epithelioid sarcoma in vitro: heterogeneity of response and the multidrug resistance phenotype. 54 61
10738220 2000
18
Immunoblot analysis of CD34 expression in histologically diverse neoplasms. 54 61
10623649 2000
19
Epithelioid sarcoma: presence of vascular-endothelial cadherin and lack of epithelial cadherin. 54 61
9839166 1998
20
Immunoreactivity for the human hematopoietic progenitor cell antigen (CD34) in lipomatous tumors. 54 61
9042286 1997
21
Vimentin-negative epithelioid sarcoma. The value of an immunohistochemical panel that includes CD34. 54 61
7679559 1993
22
The fibroma-like variant of epithelioid sarcoma. A fibrohistiocytic/myoid cell lesion often confused with benign and malignant spindle cell tumors. 54 61
1371711 1992
23
Undifferentiated carcinoma of the vulva mimicking epithelioid sarcoma. 54 61
1718178 1991
24
Cytoskeletal heterogeneity of an epithelioid sarcoma with expression of vimentin, cytokeratins, and neurofilaments. 54 61
1689556 1990
25
Multidirectional differentiation in a newly established human epithelioid sarcoma cell line (GRU-1) with co-expression of vimentin, cytokeratins and neurofilament proteins. 54 61
1688830 1990
26
Pseudomyogenic Hemangioendothelioma: Distinctive FDG PET/CT Findings With Numerous Multilayer Lesions in a Single Distal Extremity. 61
31977461 2020
27
Cutaneous Syncytial Myoepithelioma: A Nondescript Skin Tumor With Serious Diagnostic Pitfalls. 61
31587595 2020
28
Infantile Epithelioid Sarcoma with Genomic Segmental Amplification of BIRC3/YAP1 as Double Minutes Plus Trisomy 2: A Case Report. 61
31215292 2020
29
Pembrolizumab in paediatric patients with advanced melanoma or a PD-L1-positive, advanced, relapsed, or refractory solid tumour or lymphoma (KEYNOTE-051): interim analysis of an open-label, single-arm, phase 1-2 trial. 61
31812554 2020
30
Expanding the Differential of Cutaneous Epithelioid Tumors: A Case of Dedifferentiated Liposarcoma with Epithelioid Features Involving the Skin and Review of the Literature. 61
31995237 2020
31
What is new in epithelioid soft tissue tumors? 61
31686193 2020
32
Epithelioid sarcoma: half a century later. 61
31478778 2020
33
Rotational Tibio-Pelvic Constrained Hip Arthroplasty: A Surgical Technique: A Case Report. 61
31703012 2019
34
An abdominal wall mass of exogenous insulin amyloidosis in setting of metastatic sarcoma. 61
31721261 2019
35
A renal cell carcinoma with EWSR1-TFE3 fusion gene. 61
31774608 2019
36
Primary intimal epithelioid sarcoma mimicking severe atherosclerosis of aortic arch. 61
31670758 2019
37
Ipsilateral breast metastasis after axillary dissection caused by epithelioid sarcoma: a case report and pathological investigation. 61
31615564 2019
38
Spinal dural resection for oncological purposes: a systematic analysis of risks and outcomes in patients with malignant spinal tumors. 61
31628279 2019
39
Primary pleural epithelioid sarcoma of the proximal type: a diagnostic and therapeutic challenge. 61
31737506 2019
40
Classic-type epithelioid sarcoma arising from the sciatic nerve: A case report. 61
31537427 2019
41
Epithelioid sarcoma in children, adolescents, and young adults: Localized, primary metastatic and relapsed disease. Treatment results of five Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry. 61
31215116 2019
42
Soft tissue sarcomas of the upper extremities: Maximizing treatment opportunities and outcomes. 61
31404317 2019
43
CD73 complexes with emmprin to regulate MMP-2 production from co-cultured sarcoma cells and fibroblasts. 61
31510956 2019
44
Primary adrenal gland epithelioid sarcoma: A case report and literature review. 61
31087789 2019
45
[Atypical epithelioid hemangioendothelioma: a clinicopathological analysis of eight cases]. 61
31422593 2019
46
Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma of bone: Clinicopathologic features of 5 cases. 61
31233904 2019
47
Comparative Assessment of Antitumor Effects and Autophagy Induction as a Resistance Mechanism by Cytotoxics and EZH2 Inhibition in INI1-Negative Epithelioid Sarcoma Patient-Derived Xenograft. 61
31331120 2019
48
A rare case of pseudomyogenic hemangioendothelioma (PHE)/epithelioid sarcoma-like hemangioendothelioma (ES-H) of the breast first misdiagnosed as metaplastic carcinoma by FNAB and review of the literature. 61
31311568 2019
49
Magnetic resonance and computed tomography imaging features of epithelioid sarcoma in children and young adults with pathological and clinical correlation: a report from Children's Oncology Group study ARST0332. 61
30929036 2019
50
Pseudomyogenic Hemangioendothelioma. 61
30576238 2019

Variations for Epithelioid Sarcoma

Cosmic variations for Epithelioid Sarcoma:

9 (show top 50) (show all 60)
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM87902864 TP53 soft tissue,upper leg,sarcoma,NS c.641A>G p.H214R 17:7674890-7674890 6
2 COSM87935416 TP53 soft tissue,upper leg,sarcoma,NS c.499C>T p.Q167* 17:7675113-7675113 6
3 COSM90109285 SMARCB1 soft tissue,proximal,epithelioid sarcoma,NS c.796C>T p.Q266* 22:23816910-23816910 6
4 COSM92479334 SH2B3 soft tissue,upper leg,sarcoma,NS c.232G>A p.E78K 12:111418377-111418377 6
5 COSM88525472 NUP93 soft tissue,upper leg,sarcoma,NS c.116C>T p.A39V 16:56748363-56748363 6
6 COSM97107943 NRAS soft tissue,skin,sarcoma,NS c.38G>C p.G13A 1:114716123-114716123 6
7 COSM112988906 HRAS soft tissue,neck,sarcoma,NS c.37G>A p.G13S 11:534286-534286 6
8 COSM128916724 DICER1 soft tissue,proximal,epithelioid sarcoma,NS c.4859G>T p.S1620I 14:95096061-95096061 6
9 COSM87618826 ANKRD11 soft tissue,upper leg,sarcoma,NS c.2882G>T p.R961L 16:89283660-89283660 6
10 COSM101951669 soft tissue,neck,sarcoma,NS c.37G>A p.G13S 11:534286-534286 6
11 COSM106056882 soft tissue,upper leg,sarcoma,NS c.641A>G p.H214R 17:7674890-7674890 6
12 COSM111763021 soft tissue,upper leg,sarcoma,NS c.641A>G p.H214R 17:7674890-7674890 6
13 COSM144313682 soft tissue,upper leg,sarcoma,NS c.524A>G p.H175R 17:7674890-7674890 6
14 COSM105659131 soft tissue,upper leg,sarcoma,NS c.499C>T p.Q167* 17:7675113-7675113 6
15 COSM93187807 soft tissue,upper leg,sarcoma,NS c.641A>G p.H214R 17:7674890-7674890 6
16 COSM105721452 soft tissue,neck,sarcoma,NS c.37G>A p.G13S 11:534286-534286 6
17 COSM151739047 soft tissue,proximal,epithelioid sarcoma,NS c.769C>T p.Q257* 22:23816910-23816910 6
18 COSM105074075 soft tissue,proximal,epithelioid sarcoma,NS c.742C>T p.Q248* 22:23816910-23816910 6
19 COSM135372634 soft tissue,upper leg,sarcoma,NS c.116C>T p.A39V 16:56748363-56748363 6
20 COSM143375075 soft tissue,upper leg,sarcoma,NS c.524A>G p.H175R 17:7674890-7674890 6
21 COSM105624912 soft tissue,upper leg,sarcoma,NS c.641A>G p.H214R 17:7674890-7674890 6
22 COSM85813716 soft tissue,proximal,epithelioid sarcoma,NS c.631C>T p.Q211* 22:23816910-23816910 6
23 COSM122766413 soft tissue,upper leg,sarcoma,NS c.103C>T p.Q35* 17:7675113-7675113 6
24 COSM145021980 soft tissue,upper leg,sarcoma,NS c.524A>G p.H175R 17:7674890-7674890 6
25 COSM98577250 soft tissue,upper leg,sarcoma,NS c.2882G>T p.R961L 16:89283660-89283660 6
26 COSM101967324 soft tissue,neck,sarcoma,NS c.37G>A p.G13S 11:534286-534286 6
27 COSM93215390 soft tissue,upper leg,sarcoma,NS c.499C>T p.Q167* 17:7675113-7675113 6
28 COSM143182864 soft tissue,upper leg,sarcoma,NS c.22C>T p.Q8* 17:7675113-7675113 6
29 COSM144334467 soft tissue,upper leg,sarcoma,NS c.382C>T p.Q128* 17:7675113-7675113 6
30 COSM143972614 soft tissue,upper leg,sarcoma,NS c.22C>T p.Q8* 17:7675113-7675113 6
31 COSM99962766 soft tissue,proximal,epithelioid sarcoma,NS c.4859G>T p.S1620I 14:95096061-95096061 6
32 COSM112257156 soft tissue,upper leg,sarcoma,NS c.641A>G p.H214R 17:7674890-7674890 6
33 COSM142564162 soft tissue,upper leg,sarcoma,NS c.524A>G p.H175R 17:7674890-7674890 6
34 COSM144655779 soft tissue,upper leg,sarcoma,NS c.524A>G p.H175R 17:7674890-7674890 6
35 COSM142840367 soft tissue,upper leg,sarcoma,NS c.641A>G p.H214R 17:7674890-7674890 6
36 COSM122737959 soft tissue,upper leg,sarcoma,NS c.245A>G p.H82R 17:7674890-7674890 6
37 COSM144111084 soft tissue,upper leg,sarcoma,NS c.22C>T p.Q8* 17:7675113-7675113 6
38 COSM121881087 soft tissue,upper leg,sarcoma,NS c.245A>G p.H82R 17:7674890-7674890 6
39 COSM142860117 soft tissue,upper leg,sarcoma,NS c.499C>T p.Q167* 17:7675113-7675113 6
40 COSM92469558 soft tissue,proximal,epithelioid sarcoma,NS c.4859G>T p.S1620I 14:95096061-95096061 6
41 COSM91331222 soft tissue,neck,sarcoma,NS c.37G>A p.G13S 11:534286-534286 6
42 COSM144089819 soft tissue,upper leg,sarcoma,NS c.164A>G p.H55R 17:7674890-7674890 6
43 COSM144682712 soft tissue,upper leg,sarcoma,NS c.382C>T p.Q128* 17:7675113-7675113 6
44 COSM143159971 soft tissue,upper leg,sarcoma,NS c.164A>G p.H55R 17:7674890-7674890 6
45 COSM122275322 soft tissue,upper leg,sarcoma,NS c.245A>G p.H82R 17:7674890-7674890 6
46 COSM144041526 soft tissue,upper leg,sarcoma,NS c.466C>T p.Q156* 17:7675113-7675113 6
47 COSM142592590 soft tissue,upper leg,sarcoma,NS c.382C>T p.Q128* 17:7675113-7675113 6
48 COSM106084061 soft tissue,upper leg,sarcoma,NS c.499C>T p.Q167* 17:7675113-7675113 6
49 COSM131480755 soft tissue,proximal,epithelioid sarcoma,NS c.4859G>T p.S1620I 14:95096061-95096061 6
50 COSM112284809 soft tissue,upper leg,sarcoma,NS c.499C>T p.Q167* 17:7675113-7675113 6

Expression for Epithelioid Sarcoma

Search GEO for disease gene expression data for Epithelioid Sarcoma.

Pathways for Epithelioid Sarcoma

Pathways related to Epithelioid Sarcoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.84 VIM MUC1 KRT8 KRT7 CDH1
2 11.62 PECAM1 CDH5 CDH1 CD34
3 11.33 SALL4 KRT1 CDH1
4
Show member pathways
10.96 VIM KRT8 KRT7 KRT1

GO Terms for Epithelioid Sarcoma

Cellular components related to Epithelioid Sarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 intermediate filament GO:0005882 9.26 VIM KRT8 KRT7 KRT1
2 cell periphery GO:0071944 8.92 PECAM1 KRT8 CDH5 CD34

Biological processes related to Epithelioid Sarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cell morphogenesis GO:0000902 9.43 EGFR CDH5 CDH1
2 positive regulation of histone H4 acetylation GO:0090240 9.16 SMARCB1 MUC1
3 cell-cell adhesion GO:0098609 9.02 PECAM1 EGFR CDH5 CDH1 CD34
4 glomerular endothelium development GO:0072011 8.96 PECAM1 CD34

Sources for Epithelioid Sarcoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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