MCID: MYX013
MIFTS: 44

Myxofibrosarcoma

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

Aliases & Classifications for Myxofibrosarcoma

MalaCards integrated aliases for Myxofibrosarcoma:

Name: Myxofibrosarcoma 12 58 15
Fibromyxosarcoma 58 70
Dermatofibrosarcoma Protuberans, Myxoid 70
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 50 C6496
ICD10 via Orphanet 33 C49.9
UMLS via Orphanet 71 C3714524
Orphanet 58 ORPHA79105
UMLS 70 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 soft tissue sarcoma and malignant fibrous histiocytoma. An important gene associated with Myxofibrosarcoma is CREB3L2 (CAMP Responsive Element Binding Protein 3 Like 2), and among its related pathways/superpathways are PI3K-Akt signaling pathway and Pathways in cancer. The drugs Ipilimumab and Antineoplastic Agents, Immunological have been mentioned in the context of this disorder. Affiliated tissues include breast, skin and bone, and related phenotypes are cellular and immune system

Wikipedia : 73 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 314)
# Related Disease Score Top Affiliating Genes
1 soft tissue sarcoma 31.6 TP53 MDM2
2 malignant fibrous histiocytoma 31.1 TP53 SERPINA3 MUC1 MTAP MDM2 EWSR1
3 fibrous histiocytoma 30.9 SERPINA3 MTAP FUS EWSR1 CD34
4 spindle cell sarcoma 30.8 SERPINA3 MUC1 MDM2 EWSR1 CD34
5 sclerosing liposarcoma 30.8 MDM2 FUS CD34
6 ring chromosome 30.7 TP53 MDM2 EWSR1 CREB3L2
7 liposarcoma 30.7 TP53 MDM2 FUS EWSR1 CD34
8 fibrosarcoma 30.7 TP53 THBS1 FUS EWSR1 CREB3L1
9 hemangiopericytoma, malignant 30.6 SERPINA3 MUC1 MDM2 CD34
10 sarcoma 30.6 TP53 MUC1 MTAP MDM2 KRAS FUS
11 mesenchymal cell neoplasm 30.6 TP53 SERPINA3 EWSR1 CD34
12 heart sarcoma 30.6 MDM2 CD34
13 retroperitoneal sarcoma 30.6 MDM2 CD34
14 neurilemmoma 30.5 SERPINA3 MUC1 CD34
15 gastrointestinal stromal tumor 30.5 TP53 KRAS CD44 CD34 CCNA2
16 leiomyosarcoma 30.5 TP53 SERPINA3 MUC1 MDM2
17 colorectal adenocarcinoma 30.5 TP53 MUC1 KRAS CD44
18 myoepithelial carcinoma 30.4 TP53 MUC1 EWSR1
19 myxoid liposarcoma 30.4 TP53 MDM2 FUS EWSR1 CREB3L2
20 dermatofibrosarcoma protuberans 30.4 TP53 SERPINA3 CD34
21 pleomorphic lipoma 30.4 SERPINA3 MDM2 CD34
22 extraosseous osteosarcoma 30.4 MDM2 CD34
23 breast sarcoma 30.3 TP53 CD34
24 hemangioma 30.3 TP53 MUC1 KRAS CD34
25 malignant peripheral nerve sheath tumor 30.3 TP53 MUC1 MDM2 CD44 CD34
26 neurofibromatosis, type i 30.3 TP53 KRAS EWSR1 CD34
27 embryonal sarcoma 30.3 TP53 SERPINA3 MDM2
28 rhabdomyosarcoma 30.3 TP53 MDM2 KRAS FUS EWSR1 CD44
29 pleomorphic liposarcoma 30.3 SERPINA3 MUC1 MDM2 FUS EWSR1
30 connective tissue benign neoplasm 30.3 TP53 SERPINA3 MDM2 EWSR1 CD34
31 pleomorphic adenoma 30.2 TP53 MUC1 MDM2
32 myxoid chondrosarcoma 30.2 MUC1 MTAP EWSR1
33 neurofibroma 30.2 TP53 MUC1 CD34
34 chondrosarcoma 30.2 SERPINA3 MUC1 MTAP MDM2 EWSR1
35 chordoma 30.1 TP53 SERPINA3 MUC1 MTAP
36 lipomatosis, multiple 30.1 SERPINA3 MDM2 FUS EWSR1 CD34
37 lymphangioma 30.0 TP53 SERPINA3 KRAS CD34
38 angiosarcoma 30.0 TP53 MUC1 MDM2 KRAS CD34
39 cellular myxoid liposarcoma 30.0 SERPINA3 MDM2 FUS EWSR1
40 sarcoma, synovial 29.9 SERPINA3 MUC1 MDM2 FUS EWSR1 CD34
41 leukemia, acute myeloid 29.9 TP53 SERPINA3 MDM2 KRAS CD44 CD34
42 well-differentiated liposarcoma 29.8 TP53 SERPINA3 MUC1 MDM2 FUS EWSR1
43 dedifferentiated liposarcoma 29.8 TP53 SERPINA3 MUC1 MDM2 FUS EWSR1
44 ring chromosome 7 10.5 TP53 MDM2
45 actinic cheilitis 10.5 TP53 MDM2
46 esophagus verrucous carcinoma 10.5 TP53 MDM2
47 splenic diffuse red pulp small b-cell lymphoma 10.5 TP53 CCNA2
48 pancreatic foamy gland adenocarcinoma 10.5 TP53 MUC1
49 periosteal osteogenic sarcoma 10.5 TP53 MDM2
50 lip cancer 10.5 TP53 MDM2 CD44

Graphical network of the top 20 diseases related to Myxofibrosarcoma:



Diseases related to Myxofibrosarcoma

Symptoms & Phenotypes for Myxofibrosarcoma

MGI Mouse Phenotypes related to Myxofibrosarcoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cellular MP:0005384 10.15 ALDH1A1 CCNA2 CD34 CD44 CREB3L1 DCBLD2
2 immune system MP:0005387 10.1 CCNA2 CD109 CD34 CD44 DCBLD2 DCN
3 integument MP:0010771 9.91 CD109 CD34 CD44 DCN KRAS MDM2
4 liver/biliary system MP:0005370 9.76 ALDH1A1 CD44 KRAS MDM2 MTAP SKP2
5 neoplasm MP:0002006 9.5 CD34 CD44 DCN KRAS MDM2 THBS1
6 respiratory system MP:0005388 9.23 ALDH1A1 CD44 DCBLD2 DCN KRAS MTAP

Drugs & Therapeutics for Myxofibrosarcoma

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


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Ipilimumab Approved Phase 2 477202-00-9
2 Antineoplastic Agents, Immunological Phase 2
3 Immunoglobulins Phase 2
4 Antibodies Phase 2
5 Antibodies, Monoclonal 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 ENVASARC: A Pivotal Trial Of Envafolimab, And Envafolimab In Combination With Ipilimumab, In Patients With Advanced Or Metastatic Undifferentiated Pleomorphic Sarcoma Or Myxofibrosarcoma Who Have Progressed On Prior Chemotherapy Recruiting NCT04480502 Phase 2 Ipilimumab

Search NIH Clinical Center for Myxofibrosarcoma

Genetic Tests for Myxofibrosarcoma

Anatomical Context for Myxofibrosarcoma

MalaCards organs/tissues related to Myxofibrosarcoma:

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

Publications for Myxofibrosarcoma

Articles related to Myxofibrosarcoma:

(show top 50) (show all 571)
# Title Authors PMID Year
1
Potential application of genomic profiling for the diagnosis and treatment of patients with sarcoma. 61
33747210 2021
2
Pazopanib-related secondary polycythemia in metastatic myxofibrosarcoma: A case report and review of the literature. 61
32838682 2021
3
The value of re-staging chest CT at first local recurrence of extremity and trunk soft tissue sarcoma. 61
33037910 2021
4
Immunotherapy for sarcomas. 61
33611603 2021
5
Identification of a Novel MAN1A1-ROS1 Fusion Gene Through mRNA-based Screening for Tyrosine Kinase Gene Aberrations in a Patient with Leiomyosarcoma. 61
33196586 2021
6
High-intensity focused ultrasound treatment as an alternative regimen for myxofibrosarcoma. 61
33497505 2021
7
Differential diagnosis of T2 hypointense masses in musculoskeletal MRI. 61
33651128 2021
8
Remarkable response to anti-PD1 immunotherapy in refractory metastatic high-grade myxofibrosarcoma patient: A case report. 61
33761725 2021
9
Primary Sarcomas of the Larynx: A Clinicopathologic Study of 27 Cases. 61
33686585 2021
10
DDIT3 Immunohistochemistry Is a Useful Tool for the Diagnosis of Myxoid Liposarcoma. 61
32815829 2021
11
Predictors of lung recurrence and disease-specific mortality after pulmonary metastasectomy for soft tissue sarcoma. 61
33639455 2021
12
Candidate Biomarkers for Specific Intraoperative Near-Infrared Imaging of Soft Tissue Sarcomas: A Systematic Review. 61
33535618 2021
13
Metastatic Myxofibrosarcoma with Durable Response to Temozolomide Followed by Atezolizumab: A Case Report. 61
33594725 2021
14
A clinicopathologic examination of myxofibrosarcoma. Do surgical margins significantly affect local recurrence rates in this infiltrative sarcoma subtype? 61
33125727 2021
15
Gemcitabine-Containing Chemotherapy for the Treatment of Metastatic Myxofibrosarcoma Refractory to Doxorubicin: A Case Series. 61
33562486 2021
16
Broadening the spectrum of NTRK rearranged mesenchymal tumors and usefulness of pan-TRK immunohistochemistry for identification of NTRK fusions. 61
32860002 2021
17
Maxillary Sinus Myxofibrosarcoma Mimicking Nodular Fasciitis: A Rare Case Report. 61
33544385 2021
18
Establishment and characterization of NCC-MFS2-C1: a novel patient-derived cancer cell line of myxofibrosarcoma. 61
32870449 2021
19
RSF1 requires CEBP/β and hSNF2H to promote IL-1β-mediated angiogenesis: the clinical and therapeutic relevance of RSF1 overexpression and amplification in myxofibrosarcomas. 61
33496909 2021
20
Cytopathology of myxoinflammatory fibroblastic sarcoma: a series of eight cases and review of the literature. 61
33431307 2021
21
Myxofibrosarcoma: Clinical and Prognostic Value of MRI Features. 61
32729425 2021
22
Long-Term Remission with Ipilimumab/Nivolumab in Two Patients with Different Soft Tissue Sarcoma Subtypes and No PD-L1 Expression. 61
33790767 2021
23
Genome wide methylation profiling of selected matched soft tissue sarcomas identifies methylation changes in metastatic and recurrent disease. 61
33436720 2021
24
Thoracoabdominal flap reconstruction after resection of superficial soft-tissue sarcomas in the chest wall. 61
33542811 2021
25
Malignant Epithelioid Soft Tissue Tumours- A Pathologist's Perspective With Review of Literature. 61
33520482 2020
26
OSmfs: An Online Interactive Tool to Evaluate Prognostic Markers for Myxofibrosarcoma. 61
33352742 2020
27
Myxofibrosarcoma of the leg: A diagnostic challenge. 61
33363928 2020
28
Myxofibrosarcoma metastasis to the pterygopalatine fossa: A case report. 61
33145016 2020
29
Complete remission of a case of high-grade myxofibrosarcoma with lung metastases after modified MAID regimen chemotherapy. 61
32427061 2020
30
The role of surgical margin quality in myxofibrosarcoma and undifferentiated pleomorphic sarcoma. 61
33342698 2020
31
Primary cardiac myxofibrosarcoma with osteoid differentiation mimicking a left atrial myxoma: A rare entity. 61
33133322 2020
32
Efficacy of Electrochemotherapy in the Treatment of Cutaneous Melanoma Metastases and Rare Non-melanoma Skin Cancer. 61
33109587 2020
33
Combination nivolumab and bevacizumab for metastatic myxofibrosarcoma: A case report and review of the literature. 61
32905214 2020
34
Dedifferentiated liposarcoma with heterologous spindle cell rhabdomyoblastic de-differentiation: An unusual pattern expanding the morphological spectrum. 61
33154322 2020
35
Tumor-associated mortality and prognostic factors in myxofibrosarcoma - A retrospective review of 109 patients. 61
32778437 2020
36
Preoperative Evaluation of Myxofibrosarcoma: Prognostic Value and Reproducibility of Different Features on MRI. 61
32988907 2020
37
Rare Primary Malignant Bone Sarcomas. 61
33114111 2020
38
Cytopathology of myxofibrosarcoma: a study of 66 cases and literature review. 61
33041221 2020
39
MRI Features as Prognostic Factors in Myxofibrosarcoma: Proposal of MRI Grading System. 61
32928636 2020
40
Myxofibrosarcoma, in the calf of a middle aged female: a case report. 61
32794507 2020
41
Primary Osseous Low-Grade Myxofibrosarcoma of Clavicle Presenting With Multiple Skeletal Metastases. 61
33029450 2020
42
The surgical management of sarcomas of the chest wall: A 13-year single institution experience. 61
32291186 2020
43
Differential quantities of immune checkpoint-expressing CD8 T cells in soft tissue sarcoma subtypes. 61
32792357 2020
44
A Retrospective Analysis of the Efficacy of Immunotherapy in Metastatic Soft-Tissue Sarcomas. 61
32664595 2020
45
Multiple recurrent myxofibrosarcoma of the orbit: case report and review of the literature. 61
32631312 2020
46
[Myxofibrosarcoma]. 61
32974710 2020
47
Myxofibrosarcoma that Developed Rapidly in the Breast of an Elderly Man and Recurred Early after Surgery: A Case Report. 61
32602101 2020
48
SKP2 My Lou, My Darling. 61
32540853 2020
49
Periplocin mediates TRAIL-induced apoptosis and cell cycle arrest in human myxofibrosarcoma cells via the ERK/p38/JNK pathway. 61
32559583 2020
50
Retroperitoneal Myxofibrosarcoma: A Controversial Entity. 61
32345539 2020

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 ACSL5 Myxofibrosarcoma
3 39423 10 114900000 127400000 Loss DMBT1 Myxofibrosarcoma
4 42687 10 3800000 6700000 Loss TRDMT1 Myxofibrosarcoma
5 47803 10 98000000 105700000 Loss TLX1 Myxofibrosarcoma
6 47805 10 98000000 105700000 Loss PLAU Myxofibrosarcoma
7 49687 11 110000000 120700000 Loss CEP57 Myxofibrosarcoma
8 51757 11 130300000 134452384 Loss ADAMTS8 Myxofibrosarcoma
9 54382 11 43400000 48800000 Loss CD82 Myxofibrosarcoma
10 61474 12 1 14800000 Gain CCND2 Myxofibrosarcoma
11 61522 12 1 33200000 Loss LRP6 Myxofibrosarcoma
12 66186 12 26300000 33200000 Gain SSPN Myxofibrosarcoma
13 70913 12 66000000 69800000 Gain HMGA2 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 MECOM 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 217848 7 104755715 105039798 Amplificationlication SRPK2 Myxofibrosarcoma
28 217923 7 105888733 105925638 Amplificationlication NAMPT Myxofibrosarcoma
29 218114 7 107788083 108096826 Amplificationlication NRCAM Myxofibrosarcoma
30 218692 7 116312458 116438439 Amplificationlication MET Myxofibrosarcoma
31 219052 7 121513158 121702088 Amplificationlication PTPRZ1 Myxofibrosarcoma
32 219257 7 124462440 124570037 Amplificationlication POT1 Myxofibrosarcoma
33 219480 7 127292201 127732658 Amplificationlication SND1 Myxofibrosarcoma
34 219953 7 132400000 147500000 Gain ARHGEF5 Myxofibrosarcoma
35 223288 7 1855427 2272583 Amplificationlication MAD1L1 Myxofibrosarcoma
36 225548 7 47314752 47621742 Amplification TNS3 Myxofibrosarcoma
37 228044 7 73245192 73247014 Amplificationlication CLDN4 Myxofibrosarcoma
38 228772 7 81331444 81399452 Amplificationlication HGF Myxofibrosarcoma
39 229104 7 87257728 87461612 Amplificationlication RUNDC3B Myxofibrosarcoma
40 229332 7 90095737 90839904 Amplificationlication CDK14 Myxofibrosarcoma
41 229600 7 92234236 92465941 Amplificationlication CDK6 Myxofibrosarcoma
42 234953 8 140000000 146274826 Gain TG Myxofibrosarcoma
43 242385 8 7200000 43200000 Gain POLB Myxofibrosarcoma
44 262150 X 31500000 37500000 Gain OTC Myxofibrosarcoma
45 264432 X 65100000 67700000 Gain EDA Myxofibrosarcoma
46 264546 X 67700000 72200000 Gain FAM155B Myxofibrosarcoma
47 266520 X 98200000 110500000 Loss PLP1 Myxofibrosarcoma

Expression for Myxofibrosarcoma

Search GEO for disease gene expression data for Myxofibrosarcoma.

Pathways for Myxofibrosarcoma

Pathways related to Myxofibrosarcoma according to GeneCards Suite gene sharing:

(show all 24)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.75 TP53 THBS1 MDM2 KRAS CREB3L2 CREB3L1
2 12.73 TP53 SKP2 MDM2 KRAS CCNA2
3
Show member pathways
12.71 TP53 SKP2 MDM2 CD44 CCNA2
4
Show member pathways
12.69 TP53 MDM2 KRAS CREB3L2 CREB3L1
5
Show member pathways
12.43 TP53 SKP2 MDM2 CREB3L1 CCNA2
6 12.37 TP53 THBS1 MDM2 KRAS CD44
7 12.25 TP53 SKP2 MDM2 CCNA2
8
Show member pathways
12.22 TP53 MUC1 MDM2 KRAS
9 12.14 TP53 KRAS CREB3L2 CREB3L1 CCNA2
10
Show member pathways
12.08 TP53 MDM2 KRAS CREB3L2 CREB3L1
11 12.05 TP53 MDM2 KRAS CCNA2
12
Show member pathways
12.02 TP53 KRAS CREB3L2 CREB3L1
13 12 TP53 MDM2 FUS EWSR1 CCNA2
14
Show member pathways
11.99 THBS1 MUC4 MUC1 DCN
15 11.78 TP53 THBS1 MDM2 CD44
16 11.77 DCN CD44 CD34
17 11.74 TP53 THBS1 MDM2 KRAS DCN CD44
18 11.68 TP53 SKP2 MDM2 CCNA2
19
Show member pathways
11.62 TP53 MDM2 CCNA2
20 11.49 SKP2 MDM2 CCNA2
21 11.48 TP53 SKP2 MDM2 KRAS CREB3L2 CREB3L1
22 11.42 TP53 THBS1 MDM2 KRAS
23 11.37 TP53 MDM2 CCNA2
24 11.23 TP53 SKP2 MDM2 CCNA2

GO Terms for Myxofibrosarcoma

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

(show all 13)
# Name GO ID Score Top Affiliating Genes
1 positive regulation of gene expression GO:0010628 9.85 TP53 MDM2 KRAS CREB3L2 CD34
2 protein deubiquitination GO:0016579 9.81 TP53 SKP2 MDM2 CCNA2
3 Ras protein signal transduction GO:0007265 9.58 TP53 KRAS CCNA2
4 negative regulation of cysteine-type endopeptidase activity involved in apoptotic process GO:0043154 9.54 THBS1 MDM2 CD44
5 cardiac septum morphogenesis GO:0060411 9.52 TP53 MDM2
6 response to unfolded protein GO:0006986 9.5 THBS1 CREB3L2 CREB3L1
7 response to magnesium ion GO:0032026 9.49 THBS1 MDM2
8 positive regulation of transcription from RNA polymerase II promoter in response to endoplasmic reticulum stress GO:1990440 9.48 TP53 CREB3L1
9 positive regulation of transforming growth factor beta production GO:0071636 9.43 THBS1 CD34
10 cellular response to UV-C GO:0071494 9.26 TP53 MDM2
11 positive regulation of transcription from RNA polymerase II promoter in response to stress GO:0036003 9.16 TP53 MUC1
12 DNA damage response, signal transduction by p53 class mediator resulting in cell cycle arrest GO:0006977 9.13 TP53 MUC1 MDM2
13 cellular response to actinomycin D GO:0072717 8.62 TP53 MDM2

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

# Name GO ID Score Top Affiliating Genes
1 p53 binding GO:0002039 8.8 TP53 MUC1 MDM2

Sources for Myxofibrosarcoma

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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