MCID: EXT030
MIFTS: 31

Extraosseous Chondrosarcoma

Categories: Bone diseases, Cancer diseases

Aliases & Classifications for Extraosseous Chondrosarcoma

MalaCards integrated aliases for Extraosseous Chondrosarcoma:

Name: Extraosseous Chondrosarcoma 12 15
Extraskeletal Myxoid Chondrosarcoma 70

Classifications:



External Ids:

Disease Ontology 12 DOID:4549
UMLS 70 C1275278

Summaries for Extraosseous Chondrosarcoma

Disease Ontology : 12 A chondrosarcoma that is located in exclusively soft tissue.

MalaCards based summary : Extraosseous Chondrosarcoma, also known as extraskeletal myxoid chondrosarcoma, is related to extraskeletal mesenchymal chondrosarcoma and chondrosarcoma. An important gene associated with Extraosseous Chondrosarcoma is EWSR1 (EWS RNA Binding Protein 1), and among its related pathways/superpathways are Chromatin Regulation / Acetylation and Transcriptional misregulation in cancer. Affiliated tissues include exclusively soft tissue, kidney and bone, and related phenotypes are Decreased shRNA abundance (Z-score < -2) and Decreased shRNA abundance (Z-score < -2)

Related Diseases for Extraosseous Chondrosarcoma

Diseases in the Extraosseous Chondrosarcoma family:

Adult Extraosseous Chondrosarcoma

Diseases related to Extraosseous Chondrosarcoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 178)
# Related Disease Score Top Affiliating Genes
1 extraskeletal mesenchymal chondrosarcoma 32.4 TAF15 EWSR1
2 chondrosarcoma 30.6 TFG TAF15 NR4A3 EWSR1 ENO2
3 chondrosarcoma, extraskeletal myxoid 27.7 ZNF444 TFG TCF12 TAF15 SYP SSX2
4 adult extraosseous chondrosarcoma 11.2
5 bone chondrosarcoma 10.4 TAF15 NR4A3
6 glomangiosarcoma 10.3 ZNF444 SYP
7 large cell carcinoma with rhabdoid phenotype 10.3 SMARCB1 ENO2
8 laryngeal neuroendocrine tumor 10.3 SYP SMARCB1
9 ewing sarcoma of bone 10.3 EWSR1 ENO2
10 central nervous system mesenchymal non-meningothelial tumor 10.3 EWSR1 ENO2
11 cellular schwannoma 10.3 SS18 SMARCB1
12 nodular medulloblastoma 10.3 SYP SMARCB1
13 liposarcoma 10.3 TAF15 FUS EWSR1
14 plexiform schwannoma 10.3 SMARCB1 ENO2
15 sweat gland benign neoplasm 10.3 ZNF444 SMARCB1 EWSR1
16 malignant melanocytic neoplasm of the peripheral nerve sheath 10.3 SYP ENO2
17 anal neuroendocrine tumor 10.3 SYP ENO2
18 cerebral neuroblastoma 10.3 SYP ENO2
19 neuronal intestinal dysplasia, type b 10.3 SYP ENO2
20 lung meningioma 10.3 SYP ENO2
21 sinonasal undifferentiated carcinoma 10.3 SYP ENO2
22 auditory system benign neoplasm 10.3 SYP ENO2
23 papillary tumor of the pineal region 10.3 SYP ENO2
24 auditory system cancer 10.3 SYP ENO2
25 bile duct mucinous adenocarcinoma 10.3 SYP ENO2
26 nodular prostate 10.3 SYP ENO2
27 ampulla of vater benign neoplasm 10.3 SYP ENO2
28 appendix carcinoid tumor 10.3 SYP ENO2
29 carotid body cancer 10.3 SYP ENO2
30 gallbladder small cell carcinoma 10.3 SYP ENO2
31 ureter small cell carcinoma 10.3 SYP ENO2
32 keratinizing squamous cell carcinoma 10.3 SYP SMARCB1
33 cerebellar liponeurocytoma 10.3 SYP ENO2
34 middle ear adenoma 10.3 SYP ENO2
35 cerebellopontine angle primitive neuroectodermal tumor 10.3 SYP ENO2
36 urinary bladder small cell neuroendocrine carcinoma 10.3 SYP ENO2
37 gastric small cell carcinoma 10.3 SYP ENO2
38 jejunal neoplasm 10.3 SYP ENO2
39 frontal lobe neoplasm 10.3 SYP ENO2
40 sensory organ benign neoplasm 10.3 SYP ENO2
41 ovarian large-cell neuroendocrine carcinoma 10.3 SYP ENO2
42 gastric hemangioma 10.3 SYP ENO2
43 subependymoma 10.3 SYP ENO2
44 pineocytoma 10.3 SYP ENO2
45 myoblastoma 10.3 SYP ENO2
46 pineal parenchymal tumor of intermediate differentiation 10.3 SYP ENO2
47 central epithelioid sarcoma 10.3 SYP SMARCB1
48 cutaneous ganglioneuroma 10.3 SYP ENO2
49 spinal cord oligodendroglioma 10.3 SYP ENO2
50 vulvar proximal-type epithelioid sarcoma 10.3 SYP SMARCB1

Graphical network of the top 20 diseases related to Extraosseous Chondrosarcoma:



Diseases related to Extraosseous Chondrosarcoma

Symptoms & Phenotypes for Extraosseous Chondrosarcoma

GenomeRNAi Phenotypes related to Extraosseous Chondrosarcoma according to GeneCards Suite gene sharing:

26 (show all 16)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased shRNA abundance (Z-score < -2) GR00366-A-100 9.6 SMARCB1
2 Decreased shRNA abundance (Z-score < -2) GR00366-A-11 9.6 SMARCB1
3 Decreased shRNA abundance (Z-score < -2) GR00366-A-113 9.6 NFATC2
4 Decreased shRNA abundance (Z-score < -2) GR00366-A-115 9.6 DUX4L7
5 Decreased shRNA abundance (Z-score < -2) GR00366-A-122 9.6 SMARCB1
6 Decreased shRNA abundance (Z-score < -2) GR00366-A-145 9.6 NFATC2
7 Decreased shRNA abundance (Z-score < -2) GR00366-A-146 9.6 NFATC2
8 Decreased shRNA abundance (Z-score < -2) GR00366-A-177 9.6 DUX4 DUX4L7 DUX4L8
9 Decreased shRNA abundance (Z-score < -2) GR00366-A-194 9.6 NFATC2
10 Decreased shRNA abundance (Z-score < -2) GR00366-A-26 9.6 NFATC2
11 Decreased shRNA abundance (Z-score < -2) GR00366-A-34 9.6 SMARCB1
12 Decreased shRNA abundance (Z-score < -2) GR00366-A-52 9.6 NFATC2
13 Decreased shRNA abundance (Z-score < -2) GR00366-A-65 9.6 DUX4L7
14 Decreased shRNA abundance (Z-score < -2) GR00366-A-81 9.6 NFATC2
15 Decreased shRNA abundance (Z-score < -2) GR00366-A-83 9.6 SMARCB1
16 Decreased shRNA abundance (Z-score < -2) GR00366-A-85 9.6 NFATC2 SMARCB1

Drugs & Therapeutics for Extraosseous Chondrosarcoma

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Phase II Open-Label Trial of Pazopanib Administered as a Single Agent in Patients With Unresectable or Metastatic Solitary Fibrous Tumor (SFT) and Extraskeletal Myxoid Chondrosarcoma (EMC) Active, not recruiting NCT02066285 Phase 2 Pazopanib

Search NIH Clinical Center for Extraosseous Chondrosarcoma

Genetic Tests for Extraosseous Chondrosarcoma

Anatomical Context for Extraosseous Chondrosarcoma

The Foundational Model of Anatomy Ontology organs/tissues related to Extraosseous Chondrosarcoma:

19
Exclusively Soft Tissue

MalaCards organs/tissues related to Extraosseous Chondrosarcoma:

40
Kidney, Bone, Pineal, Cerebellum, Lung, Prostate, Lymph Node

Publications for Extraosseous Chondrosarcoma

Articles related to Extraosseous Chondrosarcoma:

(show top 50) (show all 363)
# Title Authors PMID Year
1
Relevance of the combination of external beam radiotherapy with the hypoxia-activated prodrug ICF05016 in an experimental model of extraskeletal myxoid chondrosarcoma. 61
32948981 2021
2
Extraskeletal Myxoid Chondrosarcoma with Molecularly Confirmed Diagnosis: A Multicenter Retrospective Study Within the Italian Sarcoma Group. 61
32572850 2021
3
ASO Author Reflections: Extraskeletal Myxoid Chondrosarcoma: A Rare Sarcoma Associated With Prolonged Survival. 61
32607606 2020
4
Locally recurrent extraskeletal myxoid chondrosarcoma of the shoulder: a case of complete neoadjuvant radiotherapy response. 61
33308312 2020
5
Extraskeletal myxoid chondrosarcoma: A high incidence of metastatic disease to lymph nodes. 61
32808356 2020
6
Extraskeletal myxoid chondrosarcoma: a case report with adjuvant intraoperative treatment. 61
33391642 2020
7
Detection of Structural Variants in Circulating Cell-Free DNA from Sarcoma Patients Using Next Generation Sequencing. 61
33287361 2020
8
Curability of patients with lymph node metastases from extremity soft-tissue sarcoma. 61
32910462 2020
9
Long-term Outcomes for Extraskeletal Myxoid Chondrosarcoma: A SEER Database Analysis. 61
32856598 2020
10
Extraskeletal Myxoid Chondrosarcoma of the Vulva Confirmed by EWSR1 FISH: A Case Report and Review of the Literature. 61
33075019 2020
11
Extraskeletal Myxoid Chondrosarcoma: State of the Art and Current Research on Biology and Clinical Management. 61
32967265 2020
12
Extraskeletal myxoid chondrosarcoma: combining cytopathology with molecular testing to achieve diagnostic accuracy. 61
32828707 2020
13
SWI/SNF complex-deficient soft tissue neoplasms: An update. 61
32646614 2020
14
Design, synthesis and evaluation of targeted hypoxia-activated prodrugs applied to chondrosarcoma chemotherapy. 61
32208207 2020
15
A renal cell carcinoma with EWSR1-TFE3 fusion gene. 61
31774608 2020
16
The Extracompartmental Tumoral Invasion of Extraskeletal Myxoid Chondrosarcoma Induces Distant Metastasis. 61
32014950 2020
17
Extraskeletal Myxoid Chondrosarcoma: Clinical and Molecular Characteristics and Outcomes of Patients Treated at Two Institutions. 61
32612944 2020
18
Extraskeletal Myxoid Chondrosarcoma: Long-Term Survival in the Setting of Metastatic Disease. 61
32963861 2020
19
Results of a prospective phase 2 study of pazopanib in patients with surgically unresectable or metastatic chondrosarcoma. 61
31509242 2020
20
Apatinib for Treatment of Inoperable Metastatic or Locally Advanced Chondrosarcoma: What We Can Learn About the Biological Behavior of Chondrosarcoma from a Two-Center Study. 61
32547189 2020
21
NR4A3 Immunohistochemistry Lacks Sensitivity for the Diagnosis of Extraskeletal Myxoid Chondrosarcoma. 61
31414988 2019
22
Extraskeletal Myxoid Chondrosarcoma of the Midfoot Presenting as Charcot Arthropathy: A Case Report. 61
31589174 2019
23
Pazopanib for treatment of advanced extraskeletal myxoid chondrosarcoma: a multicentre, single-arm, phase 2 trial. 61
31331701 2019
24
NR4A3 fusion proteins trigger an axon guidance switch that marks the difference between EWSR1 and TAF15 translocated extraskeletal myxoid chondrosarcomas. 61
31020999 2019
25
EWSR1-NFATC2 Translocation-associated Sarcoma Clinicopathologic Findings in a Rare Aggressive Primary Bone or Soft Tissue Tumor. 61
30994538 2019
26
Extraskeletal Myxoid Chondrosarcoma of the Parotid Gland. 61
31909032 2019
27
A 5-year follow-up of vulval swelling due to extraskeletal myxoid chondrosarcoma: A rare case report. 61
30967942 2019
28
Extraskeletal myxoid chondrosarcoma of the leg in a child: A case report. 61
30985717 2019
29
A rare manifestation of extraskeletal myxoid chondrosarcoma with a huge expanding hematoma. 61
28187993 2019
30
Intraluminal EWSR1-CREB1 gene rearranged, low-grade myxoid sarcoma of the pulmonary artery resembling extraskeletal myxoid chondrosarcoma (EMC). 61
30326150 2019
31
Ewing's sarcoma/peripheral primitive neuroectodermal tumor with extraskeletal myxoid chondrosarcoma-like areas: a case report. 61
31933786 2019
32
Efficacy and Safety of Nanosomal Docetaxel Lipid Suspension-Based Chemotherapy in Sarcoma: A Multicenter, Retrospective Study. 61
31827370 2019
33
Fine-needle aspiration features of extraskeletal myxoid chondrosarcoma: A study of cytological and molecular features. 61
30353688 2018
34
Systemic manifestations of extraskeletal myxoid chondrosarcoma associated with a novel t(2;22)(q34;q12) EWS translocation in a child and a review of the literature. 61
30776935 2018
35
Well-differentiated extraskeletal chondrosarcoma: about a new case. 61
30546146 2018
36
Molecular and Clinicopathologic Heterogeneity of Intracranial Tumors Mimicking Extraskeletal Myxoid Chondrosarcoma. 61
29924341 2018
37
Benefit of Radiotherapy in Extraskeletal Myxoid Chondrosarcoma: A Propensity Score Weighted Population-based Analysis of the SEER Database. 61
27819877 2018
38
Identification of an Actionable Mutation of KIT in a Case of Extraskeletal Myxoid Chondrosarcoma. 61
29937513 2018
39
[Primary extraskeletal myxoid chondrosarcoma of the corpus callosum: report of a case]. 61
29886600 2018
40
Extra-axial chordoma: a clinicopathologic analysis of six cases. 61
29560513 2018
41
Extraskeletal Chondrosarcoma: Long-term Follow-up of a Patient with Metastatic Disease. 61
30065902 2018
42
INSM1 expression and its diagnostic significance in extraskeletal myxoid chondrosarcoma. 61
29327709 2018
43
Pre- and postoperative radiotherapy for extremity soft tissue sarcoma: Evaluation of inter-observer target volume contouring variability among French sarcoma group radiation oncologists. 61
29545115 2018
44
Comprehensive high-resolution genomic profiling and cytogenetics of human chondrocyte cultures by GTG-banding, locus-specific FISH, SKY and SNP array. 61
29683471 2018
45
Extraskeletal Myxoid Chondrosarcoma of Nasopharynx: An Oncologic Entity Rarely Reported. 61
29657686 2018
46
Extraosseous Intradural Chondrosarcoma of the Cervical Spine: A Case Report with Brief Review of Literature. 61
29682386 2018
47
Extraskeletal Myxoid Chondrosarcoma: A Comparative Study of Imaging and Pathology. 61
29977924 2018
48
Myxoid chondrosarcoma extraskeletal in pelvis location : a case report. 61
30325000 2018
49
Extraskeletal myxoid chondrosarcoma with massive pulmonary metastases. 61
30534357 2018
50
Myxoid and reticular angiomatoid fibrous histiocytoma: a case confirmed by fluorescence in situ hybridization analysis for EWSR1 rearrangement. 61
31938448 2018

Variations for Extraosseous Chondrosarcoma

Expression for Extraosseous Chondrosarcoma

Search GEO for disease gene expression data for Extraosseous Chondrosarcoma.

Pathways for Extraosseous Chondrosarcoma

Pathways related to Extraosseous Chondrosarcoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 11.76 TAF15 SS18 SMARCB1 FUS EWSR1
2 11.45 TAF15 SSX2 SS18 NR4A3 FUS EWSR1

GO Terms for Extraosseous Chondrosarcoma

Cellular components related to Extraosseous Chondrosarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 nucleus GO:0005634 9.97 ZNF444 TCF12 TAF15 SSX2 SS18 SMARCB1
2 SWI/SNF complex GO:0016514 8.96 SS18 SMARCB1
3 npBAF complex GO:0071564 8.62 SS18 SMARCB1

Biological processes related to Extraosseous Chondrosarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 positive regulation of transcription by RNA polymerase II GO:0045944 9.8 TCF12 SS18 SMARCB1 NR4A3 NFATC2 ETV1
2 regulation of transcription, DNA-templated GO:0006355 9.76 TAF15 SSX2 PATZ1 NR4A3 NFATC2 FUS
3 regulation of transcription by RNA polymerase II GO:0006357 9.61 ZNF444 TCF12 SMARCB1 PATZ1 NR4A3 NFATC2
4 positive regulation of transcription, DNA-templated GO:0045893 9.17 TCF12 TAF15 SS18 PATZ1 NFATC2 FUS

Molecular functions related to Extraosseous Chondrosarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 sequence-specific double-stranded DNA binding GO:1990837 9.72 ZNF444 TCF12 NFATC2 ETV1 DUX4
2 RNA polymerase II proximal promoter sequence-specific DNA binding GO:0000978 9.7 ZNF444 TCF12 PATZ1 NR4A3 NFATC2 ETV1
3 DNA-binding transcription activator activity, RNA polymerase II-specific GO:0001228 9.65 TCF12 NR4A3 NFATC2 ETV1 DUX4
4 cAMP response element binding GO:0035497 9.37 TCF12 NR4A3
5 DNA binding GO:0003677 9.32 ZNF444 TCF12 TAF15 SMARCB1 PATZ1 NR4A3
6 transcription coregulator activity GO:0003712 9.26 TAF15 SMARCB1 FUS EWSR1

Sources for Extraosseous Chondrosarcoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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