MCID: MYX013
MIFTS: 38

Myxofibrosarcoma

Categories: Cancer diseases, Neuronal diseases, Rare diseases, Skin diseases

Aliases & Classifications for Myxofibrosarcoma

MalaCards integrated aliases for Myxofibrosarcoma:

Name: Myxofibrosarcoma 12 58
Fibromyxosarcoma 58 71
Dermatofibrosarcoma Protuberans, Myxoid 71
Myxoid Malignant Fibrous Histiocytoma 58
Fibromyxoid Sarcoma 12

Characteristics:

Orphanet epidemiological data:

58
myxofibrosarcoma
Inheritance: Not applicable; Age of onset: Adult;

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

Disease Ontology 12 DOID:0080534
NCIt 49 C6496
ICD10 via Orphanet 33 C49.9
UMLS via Orphanet 72 C3714524
Orphanet 58 ORPHA79105
UMLS 71 C0334454 C3714524

Summaries for Myxofibrosarcoma

Disease Ontology : 12 A sarcoma that arises from the soft tissue and is characterized by the presence of spindle-shaped cells, cellular pleomorphism, thin-walled blood vessels, fibrous septa, and myxoid stroma.

MalaCards based summary : Myxofibrosarcoma, also known as fibromyxosarcoma, is related to spindle cell sarcoma and fibrous histiocytoma. An important gene associated with Myxofibrosarcoma is CREB3L1 (CAMP Responsive Element Binding Protein 3 Like 1), and among its related pathways/superpathways are PI3K-Akt signaling pathway and Mitotic G1-G1/S phases. The drugs Sunitinib and Sorafenib have been mentioned in the context of this disorder. Affiliated tissues include bone, breast and heart.

Wikipedia : 74 A histiocytoma is a tumour consisting of histiocytes. Histiocytes are cells that are a part of the... more...

Related Diseases for Myxofibrosarcoma

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

(show top 50) (show all 152)
# Related Disease Score Top Affiliating Genes
1 spindle cell sarcoma 30.9 S100B DES
2 fibrous histiocytoma 30.7 S100B FUS DES
3 ossifying fibromyxoid tumor 30.7 S100B DES
4 hemangiopericytoma, malignant 30.6 S100B DES
5 embryonal sarcoma 30.2 S100B DES
6 rhabdomyosarcoma 30.2 S100B MET DES
7 gastrointestinal stromal tumor 29.9 S100B DES CCNA2
8 pleomorphic liposarcoma 29.9 S100B FUS DES
9 lipomatosis, multiple 29.9 FUS DES
10 chordoma 29.9 S100B MET DES
11 neurilemmoma 29.8 S100B DES
12 sarcoma 29.5 S100B MET FUS CREB3L2
13 extraosseous chondrosarcoma 29.4 FUS CREB3L2
14 sarcoma, synovial 29.4 S100B MET FUS DES
15 chondrosarcoma, extraskeletal myxoid 29.3 FUS CREB3L2
16 undifferentiated pleomorphic sarcoma 10.7
17 malignant fibroxanthoma 10.6
18 histiocytoma 10.6
19 soft tissue sarcoma 10.5
20 fibrosarcoma 10.4
21 liposarcoma 10.4
22 doxorubicin induced cardiomyopathy 10.3 MET DES
23 myxoid liposarcoma 10.3
24 malignant triton tumor 10.3 S100B DES
25 cutaneous leiomyosarcoma 10.3 S100B DES
26 syringocystadenoma papilliferum 10.2 S100B DES
27 mesenchymal cell neoplasm 10.2
28 ring chromosome 10.2
29 spindle cell rhabdomyosarcoma 10.2 S100B DES
30 ovarian small cell carcinoma 10.2 S100B DES
31 dermatofibrosarcoma protuberans 10.2
32 syringoma 10.2 S100B DES
33 perivascular epithelioid cell tumor 10.2 S100B DES
34 cutaneous fibrous histiocytoma 10.2 S100B DES
35 dermoid cyst 10.2
36 cystic teratoma 10.2
37 mature teratoma 10.2
38 sclerosing liposarcoma 10.2
39 papular mucinosis 10.2
40 retroperitoneal sarcoma 10.2
41 kidney sarcoma 10.2
42 connective tissue benign neoplasm 10.1
43 dedifferentiated liposarcoma 10.1
44 leiomyosarcoma 10.1
45 rare tumor 10.1
46 granular cell tumor 10.1 S100B DES
47 thrombocytopenia 10.1
48 adenocarcinoma 10.1
49 neuroblastoma 10.1
50 polycythemia 10.1

Graphical network of the top 20 diseases related to Myxofibrosarcoma:



Diseases related to Myxofibrosarcoma

Symptoms & Phenotypes for Myxofibrosarcoma

Drugs & Therapeutics for Myxofibrosarcoma

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

(show all 12)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Sunitinib Approved, Investigational Phase 2 557795-19-4, 341031-54-7 5329102
2
Sorafenib Approved, Investigational Phase 2 284461-73-0 216239 406563
3
Pembrolizumab Approved Phase 2 1374853-91-4
4
Atezolizumab Approved, Investigational Phase 2 1380723-44-3
5 Angiogenesis Inhibitors Phase 2
6 Protein Kinase Inhibitors Phase 2
7 Immunosuppressive Agents Phase 2
8 Antineoplastic Agents, Immunological Phase 2
9 Antibodies, Monoclonal Phase 2
10 Immunologic Factors Phase 2
11 Antibodies Phase 2
12 Immunoglobulins Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Phase II Study of MLN8237 in Advanced/Metastatic Sarcoma Completed NCT01653028 Phase 2 Alisertib
2 A Multicenter Phase II Study of Continuous Dosing of Sunitinib (Sutent®, SU11248) in Non-GIST Sarcomas Completed NCT00474994 Phase 2 sunitinib malate
3 A Multicenter Phase II Study of Sorafenib (BAY43-9006) in Non-GIST Sarcomas Completed NCT00245102 Phase 2 sorafenib tosylate
4 SU2C-SARC032: A Phase II Randomized Controlled Trial of Neoadjuvant Pembrolizumab With Radiotherapy and Adjuvant Pembrolizumab in Patients With High-Risk, Localized Soft Tissue Sarcoma of the Extremity Recruiting NCT03092323 Phase 2 Pembrolizumab
5 A European, Multicenter, Randomized, Open-label, Phase II Trial Aiming to Assess the Clinical and Biological Activity of an Anti-PD-L1 (Atezolizumab) in Operable Localised Soft Tissue Sarcomas Patients to be Treated With Radiotherapy Recruiting NCT03474094 Phase 2
6 Multimodal Immune Characterization of RAre Soft Tissue Sarcoma - MIRAS Project From SARRA (SARcome RAre) Project of the French Sarcoma Group Not yet recruiting NCT03967834

Search NIH Clinical Center for Myxofibrosarcoma

Genetic Tests for Myxofibrosarcoma

Anatomical Context for Myxofibrosarcoma

MalaCards organs/tissues related to Myxofibrosarcoma:

40
Bone, Breast, Heart, Brain, Lung, Skin, Prostate

Publications for Myxofibrosarcoma

Articles related to Myxofibrosarcoma:

(show top 50) (show all 497)
# Title Authors PMID Year
1
Comparative Study of Myxofibrosarcoma With Undifferentiated Pleomorphic Sarcoma: Histopathologic and Clinicopathologic Review. 61
31651522 2020
2
Peptide vaccinations elicited strong immune responses that were reboosted by anti-PD1 therapy in a patient with myxofibrosarcoma. 61
31853575 2019
3
Atypical lipomatous tumour/well-differentiated liposarcoma and de-differentiated liposarcoma in patients aged ≤ 40 years: a study of 116 patients. 61
31471922 2019
4
Systematic screening identifies a 2-gene signature as a high-potential prognostic marker of undifferentiated pleomorphic sarcoma/myxofibrosarcoma. 61
31742892 2019
5
Primary low grade myxofibrosarcoma of the liver with benign presentation but malignant outcome: a case report. 61
31718576 2019
6
Seven-year follow-up of spontaneous bone regeneration following segmental mandibulectomy: Alternative option for mandibular reconstruction. 61
31803391 2019
7
Prognostic Impact of CD44 Expression in Patients With Myxofibrosarcoma. 61
31662543 2019
8
Proteomic research in sarcomas - current status and future opportunities. 61
31722230 2019
9
Small soft tissue masses indeterminate at imaging: histological diagnoses at a tertiary orthopedic oncology clinic. 61
30903259 2019
10
Does pre-operative MRI predict the risk of local recurrence in primary myxofibrosarcoma of the extremities? 61
31111597 2019
11
Upper extremity myxofibrosarcoma mimicking an erosive inflammatory arthritis: a case report. 61
30989248 2019
12
Successful multidisciplinary clinical approach and molecular characterization by whole transcriptome sequencing of a cardiac myxofibrosarcoma: A case report. 61
31624749 2019
13
What is an adequate margin for infiltrative soft-tissue sarcomas? 61
31627932 2019
14
Spindle cell liposarcoma with a TRIO-TERT fusion transcript. 61
30793229 2019
15
Thermal Imaging Facilitates Design of a Keystone Perforator Island Flap for a Myxofibrosarcoma Resection Reconstruction: Case Report. 61
31592380 2019
16
Assessment of the platelet-derived growth factor receptor alpha antibody olaratumab in a panel of patient-derived soft tissue sarcoma xenografts. 61
31331295 2019
17
Identification of infectious species after resection of soft-tissue sarcomas. 61
30816563 2019
18
Establishment and Characterization of Histologically and Molecularly Stable Soft-tissue Sarcoma Xenograft Models for Biological Studies and Preclinical Drug Testing. 61
30962320 2019
19
Prognostic Factors and Outcomes for Patients With Myxofibrosarcoma: A 13-Year Retrospective Evaluation. 61
31177139 2019
20
Surgical Reconstruction after Resection of a Large Myxofibrosarcoma of the Upper Extremity. 61
31363294 2019
21
MAGE-A3 is a Clinically Relevant Target in Undifferentiated Pleomorphic Sarcoma/Myxofibrosarcoma. 61
31096717 2019
22
Spontaneous Regression of Myxofibrosarcoma of the Thigh after Open Biopsy. 61
31182952 2019
23
CBX3 predicts an unfavorable prognosis and promotes tumorigenesis in osteosarcoma. 61
30942427 2019
24
Radium-223 in combination with paclitaxel in cancer patients with bone metastases: safety results from an open-label, multicenter phase Ib study. 61
30547207 2019
25
Comprehensive Genomic Profiling of Adult Renal Sarcomas Provides Insight into Disease Biology and Opportunities for Targeted Therapies. 61
31412008 2019
26
Establishment and characterization of a novel cell line, NCC-MFS1-C1, derived from a patient with myxofibrosarcoma. 61
30737712 2019
27
Aggressive invasive recurrence of a right atrial myxofibrosarcoma. 61
30851052 2019
28
Emergency Ross Procedure for Pediatric Aortic Valve Myxofibrosarcoma. 61
30266618 2019
29
Malignant solitary fibrous tumor in the subcutis: Report of a rare superficial malignant type and review of published work. 61
30614065 2019
30
Non-islet-cell tumour hypoglycaemia (NICTH): About a series of 6 cases. 61
29555080 2019
31
Immunotherapy for advanced lung cancer combined with surgery for mediastinal myxofibrosarcoma: a case report. 61
30806844 2019
32
Spontaneous retroperitoneal liposarcoma in a free-ranging juvenile golden-headed lion tamarin (Leontopithecus chrysomelas). 61
30230546 2019
33
Identification of novel GNAS mutations in intramuscular myxoma using next-generation sequencing with single-molecule tagged molecular inversion probes. 61
30736805 2019
34
Soft-Tissue Sarcoma Masquerading as a Haematoma. 61
31031537 2019
35
Psychosocial Distress Scores and Needs among Newly Diagnosed Sarcoma Patients: A Provincial Experience. 61
31354383 2019
36
A rare case of TLE1-positive sclerosing epithelioid fibrosarcoma expanding the differential diagnosis of TLE1-positive tumors: a case report. 61
31020273 2019
37
Survival Outcome and Prognostic Factors After Pulmonary Metastasectomy in Sarcoma Patients: A 18-Year Experience at a Single High-volume Referral Center. 61
30048248 2019
38
Identification Of Actionable Genetic Targets In Primary Cardiac Sarcomas. 61
31807008 2019
39
High Recurrence Rate of Myxofibrosarcoma: The Effect of Radiotherapy Is Not Clear. 61
31662702 2019
40
Palliative Surgical Resection of an Extensive Metastatic Cardiac Myxofibrosarcoma. 61
30967750 2019
41
An entire clinical course of mediastinal myxofibrosarcoma treated by surgery and radiation. 61
29748938 2018
42
Impact of perioperative chemotherapy and radiotherapy in patients with primary extremity soft tissue sarcoma: retrospective analysis across major histological subtypes and major reference centres. 61
30384013 2018
43
Subclassification of pleomorphic sarcomas: How and why should we care? 61
30340082 2018
44
Fine-needle aspiration features of extraskeletal myxoid chondrosarcoma: A study of cytological and molecular features. 61
30353688 2018
45
Orbital Myxofibrosarcoma: Case Report and Review of Literature. 61
30204636 2018
46
[Leptomeningeal metastasis of myxofibrosarcoma: a case report]. 61
30481943 2018
47
Clinical significance of the tail-like pattern in soft-tissue sarcomas on magnetic resonance imaging. 61
30017714 2018
48
A combination of irinotecan/cisplatinum and irinotecan/temozolomide or tumor-targeting Salmonella typhimurium A1-R arrest doxorubicin- and temozolomide-resistant myxofibrosarcoma in a PDOX mouse model. 61
30292411 2018
49
Localized Myxofibrosarcomas: Roles of Surgical Margins and Adjuvant Radiation Therapy. 61
30191871 2018
50
New observations in tumor cell plasticity: mutational profiling in a case of metastatic melanoma with biphasic sarcomatoid transdifferentiation. 61
29785541 2018

Variations for Myxofibrosarcoma

Copy number variations for Myxofibrosarcoma from CNVD:

7 (show all 47)
# CNVD ID Chromosome Start End Type Gene Symbol CNVD Disease
1 29705 1 234600000 241700000 Loss AKT3 Myxofibrosarcoma
2 38875 10 105700000 114900000 Loss FACL5 Myxofibrosarcoma
3 39423 10 114900000 127400000 Loss DMBT1 Myxofibrosarcoma
4 42687 10 3800000 6700000 Loss DNMT2 Myxofibrosarcoma
5 47803 10 98000000 105700000 Loss HOX11 Myxofibrosarcoma
6 47805 10 98000000 105700000 Loss PLAU Myxofibrosarcoma
7 49687 11 110000000 120700000 Loss PIG8 Myxofibrosarcoma
8 51757 11 130300000 134452384 Loss ADAMTS8 Myxofibrosarcoma
9 54382 11 43400000 48800000 Loss KAI1 Myxofibrosarcoma
10 61474 12 1 14800000 Gain CCND2 Myxofibrosarcoma
11 61522 12 1 33200000 Loss LRP6 Myxofibrosarcoma
12 66186 12 26300000 33200000 Gain KRAG Myxofibrosarcoma
13 70913 12 66000000 69800000 Gain HMGIC Myxofibrosarcoma
14 73112 12 91200000 94800000 Loss BTG1 Myxofibrosarcoma
15 108897 17 23200000 35400000 Loss LIG3 Myxofibrosarcoma
16 115128 17 54900000 59900000 Gain TBX2 Myxofibrosarcoma
17 124176 19 1 6900000 Gain SH3GL1 Myxofibrosarcoma
18 143681 2 23900000 31900000 Loss ALK Myxofibrosarcoma
19 147727 2 69800000 136600000 Loss CXCR4 Myxofibrosarcoma
20 148303 2 75400000 83700000 Loss REG1A Myxofibrosarcoma
21 151117 20 17800000 27100000 Loss JAG1 Myxofibrosarcoma
22 170894 3 161200000 184200000 Loss MDS1 Myxofibrosarcoma
23 177567 3 58500000 63700000 Loss FHIT Myxofibrosarcoma
24 186568 4 36900000 114100000 Loss IBSP Myxofibrosarcoma
25 187541 4 50700000 52400000 Gain AFP Myxofibrosarcoma
26 208064 6 164400000 170899992 Loss PDCD2 Myxofibrosarcoma
27 217923 7 105888733 105925638 Amplificationlication PBEF1 Myxofibrosarcoma
28 218114 7 107788083 108096826 Amplificationlication NRCAM Myxofibrosarcoma
29 218692 7 116312458 116438439 Amplificationlication MET Myxofibrosarcoma
30 219052 7 121513158 121702088 Amplificationlication PTPRZ1 Myxofibrosarcoma
31 219257 7 124462440 124570037 Amplificationlication POT1 Myxofibrosarcoma
32 219953 7 132400000 147500000 Gain TIM1 Myxofibrosarcoma
33 223288 7 1855427 2272583 Amplificationlication MAD1L1 Myxofibrosarcoma
34 225548 7 47314752 47621742 Amplification TNS3 Myxofibrosarcoma
35 228044 7 73245192 73247014 Amplificationlication CLDN4 Myxofibrosarcoma
36 228772 7 81331444 81399452 Amplificationlication HGF Myxofibrosarcoma
37 229104 7 87257728 87461612 Amplificationlication RPIP9 Myxofibrosarcoma
38 229332 7 90095737 90839904 Amplificationlication PFTK1 Myxofibrosarcoma
39 229600 7 92234236 92465941 Amplificationlication CDK6 Myxofibrosarcoma
40 234953 8 140000000 146274826 Gain TG Myxofibrosarcoma
41 242385 8 7200000 43200000 Gain POLB Myxofibrosarcoma
42 262150 X 31500000 37500000 Gain OTC Myxofibrosarcoma
43 264432 X 65100000 67700000 Gain ED1 Myxofibrosarcoma
44 264546 X 67700000 72200000 Gain TED Myxofibrosarcoma
45 266520 X 98200000 110500000 Loss PLP Myxofibrosarcoma
46 219480 7 127292201 127732658 Amplificationlication SND1 Myxofibrosarcoma
47 217848 7 104755715 105039798 Amplificationlication SRPK2 Myxofibrosarcoma

Expression for Myxofibrosarcoma

Search GEO for disease gene expression data for Myxofibrosarcoma.

Pathways for Myxofibrosarcoma

GO Terms for Myxofibrosarcoma

Biological processes related to Myxofibrosarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 endoplasmic reticulum unfolded protein response GO:0030968 8.62 CREB3L2 CREB3L1

Molecular functions related to Myxofibrosarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 9.35 SKP2 S100B MET FUS DES
2 cAMP response element binding GO:0035497 8.62 CREB3L2 CREB3L1

Sources for Myxofibrosarcoma

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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