MCID: MYX013
MIFTS: 39

Myxofibrosarcoma

Categories: Skin diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Myxofibrosarcoma

MalaCards integrated aliases for Myxofibrosarcoma:

Name: Myxofibrosarcoma 55
Fibromyxosarcoma 55 69
Dermatofibrosarcoma Protuberans, Myxoid 69
Myxoid Malignant Fibrous Histiocytoma 55

Characteristics:

Orphanet epidemiological data:

55
myxofibrosarcoma
Inheritance: Not applicable; Age of onset: Adult;

Classifications:

Orphanet: 55  
Rare neurological diseases


External Ids:

Orphanet 55 ORPHA79105
UMLS via Orphanet 70 C3714524
ICD10 via Orphanet 33 C49.9

Summaries for Myxofibrosarcoma

MalaCards based summary : Myxofibrosarcoma, also known as fibromyxosarcoma, is related to dermatofibrosarcoma protuberans 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 Kaposi's sarcoma-associated herpesvirus infection and Viral carcinogenesis. The drugs Doxorubicin and Cyclophosphamide have been mentioned in the context of this disorder. Affiliated tissues include heart, bone and brain.

Wikipedia : 72 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 106)
# Related Disease Score Top Affiliating Genes
1 dermatofibrosarcoma protuberans 29.6 DES S100B
2 fibrous histiocytoma 29.5 DES FUS
3 malignant triton tumor 10.4 DES S100B
4 juvenile xanthogranuloma 10.4 DES S100B
5 cutaneous fibrous histiocytoma 10.4 DES S100B
6 pulmonary vein stenosis 10.4 DES MKI67
7 granular cell tumor 10.4 DES S100B
8 adenosarcoma 10.4 DES MKI67
9 hidradenocarcinoma 10.3 MKI67 S100B
10 spindle cell thymoma 10.3 DES MUC1
11 parachordoma 10.3 DES MUC1
12 odontogenic myxoma 10.3 CD36 S100B
13 spindle cell rhabdomyosarcoma 10.3 DES MUC1
14 biphasic synovial sarcoma 10.3 DES MUC1
15 localized hypertrophic neuropathy 10.3 MUC1 S100B
16 sclerosing perineurioma 10.3 MUC1 S100B
17 intracranial cysts 10.3 MUC1 S100B
18 reticular perineurioma 10.3 MUC1 S100B
19 non-functioning pituitary adenoma 10.3 MUC1 S100B
20 epithelioid malignant peripheral nerve sheath tumor 10.3 MUC1 S100B
21 fibrous meningioma 10.3 MUC1 S100B
22 chordoid meningioma 10.3 MUC1 S100B
23 eccrine porocarcinoma 10.3 MUC1 S100B
24 ischemic fasciitis 10.3 DES MUC1
25 aggressive digital papillary adenocarcinoma 10.3 MUC1 S100B
26 plasmablastic lymphoma 10.3 DES MUC1
27 proximal spinal muscular atrophy 10.3 DES MUC1
28 sinonasal undifferentiated carcinoma 10.3 MKI67 MUC1
29 chordoid glioma 10.3 MUC1 S100B
30 perivascular epithelioid cell tumor 10.3 DES MUC1
31 myxopapillary ependymoma 10.3 MUC1 S100B
32 monophasic synovial sarcoma 10.3 MUC1 S100B
33 reticulum cell sarcoma 10.3 DES MUC1
34 anaplastic ependymoma 10.3 MUC1 S100B
35 mesenchymal chondrosarcoma 10.3 DES MUC1
36 endometrial mucinous adenocarcinoma 10.3 MKI67 MUC1
37 neurilemmoma 10.3 DES S100B
38 perineurioma 10.2 MUC1 S100B
39 spiradenoma 10.2 MUC1 S100B
40 papilloma of choroid plexus 10.2 MUC1 S100B
41 functioning pituitary adenoma 10.2 MUC1 S100B
42 meningeal melanocytoma 10.2 MUC1 S100B
43 dendritic cell tumor 10.2 MUC1 S100B
44 cerebral primitive neuroectodermal tumor 10.2 MUC1 S100B
45 benign mesothelioma 10.2 DES MUC1
46 sarcoma 10.2
47 intraneural perineurioma 10.2 MUC1 S100B
48 papillary adenocarcinoma 10.2 MUC1 S100B
49 fasciitis 10.2 DES MUC1
50 embryonal sarcoma 10.2 DES MKI67 S100B

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 13)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Doxorubicin Approved, Investigational Phase 2, Phase 3,Phase 1 23214-92-8 31703
2
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
3
Ifosfamide Approved Phase 2, Phase 3 3778-73-2 3690
4
Mechlorethamine Approved, Investigational Phase 2, Phase 3 51-75-2 4033
5
Doxil Approved June 1999 Phase 2, Phase 3,Phase 1 31703
6 Isophosphamide mustard Phase 2, Phase 3 0
7 Alkylating Agents Phase 2, Phase 3
8 Topoisomerase Inhibitors Phase 2, Phase 3,Phase 1
9 Anti-Bacterial Agents Phase 2, Phase 3,Phase 1
10 Antibiotics, Antitubercular Phase 2, Phase 3,Phase 1
11 razoxane Phase 2
12 Protective Agents Phase 2
13 Antimitotic Agents Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Radiation Therapy With or Without Combination Chemotherapy or Pazopanib Hydrochloride Before Surgery in Treating Patients With Newly Diagnosed Non-rhabdomyosarcoma Soft Tissue Sarcomas That Can Be Removed by Surgery Recruiting NCT02180867 Phase 2, Phase 3 Doxorubicin Hydrochloride;Ifosfamide;Pazopanib Hydrochloride
2 Alisertib in Treating Patients With Advanced or Metastatic Sarcoma Completed NCT01653028 Phase 2 Alisertib
3 Sapanisertib or Pazopanib Hydrochloride in Treating Patients With Locally Advanced or Metastatic Sarcoma Recruiting NCT02601209 Phase 1, Phase 2 Pazopanib Hydrochloride;Sapanisertib
4 Doxorubicin With Upfront Dexrazoxane Plus Olaratumab for the Treatment of Advanced or Metastatic Soft Tissue Sarcoma Recruiting NCT02584309 Phase 2 Dexrazoxane;Doxorubicin;Olaratumab
5 Ribociclib and Doxorubicin in Treating Patients With Metastatic or Advanced Soft Tissue Sarcomas That Cannot Be Removed by Surgery Recruiting NCT03009201 Phase 1 Doxorubicin;Ribociclib

Search NIH Clinical Center for Myxofibrosarcoma

Genetic Tests for Myxofibrosarcoma

Anatomical Context for Myxofibrosarcoma

MalaCards organs/tissues related to Myxofibrosarcoma:

38
Heart, Bone, Brain, Skin, Lung, Breast, Prostate

Publications for Myxofibrosarcoma

Articles related to Myxofibrosarcoma:

(show top 50) (show all 193)
# Title Authors Year
1
Biology and Management of Undifferentiated Pleomorphic Sarcoma, Myxofibrosarcoma, and Malignant Peripheral Nerve Sheath Tumors: State of the Art and Perspectives. ( 29220302 )
2018
2
Successful management of a rare type of complex myxofibrosarcoma which affected right lung and invading into the left atrium under the guidance of multimode image. ( 29409129 )
2018
3
Primary myxofibrosarcoma of the kidney. ( 29075931 )
2018
4
Myxofibrosarcoma of unusual sites. ( 29065228 )
2018
5
Left atrial appendage myxofibrosarcoma: A rare masquerader of myxoma and thrombus-"all that glitters is not gold". ( 29280521 )
2018
6
Pembrolizumab for Refractory Metastatic Myxofibrosarcoma: A Case Report. ( 29361819 )
2018
7
Myxofibrosarcoma: First report of myxofibrosarcoma of bone arising at a bone infarct. ( 28429048 )
2017
8
High-grade myxofibrosarcoma of the abdominal wall. ( 28576911 )
2017
9
Low grade myxofibrosarcoma in the right ventricle presenting as pulmonary thromboembolism. ( 29312771 )
2017
10
Discoidin, CUB and LCCL domain-containing protein 2 (DCBLD2) is a novel biomarker of myxofibrosarcoma invasion identified by global protein expression profiling. ( 28668639 )
2017
11
Establishment of a novel cellular model for myxofibrosarcoma heterogeneity. ( 28304377 )
2017
12
Myxofibrosarcoma primary cultures: molecular and pharmacological profile. ( 29449896 )
2017
13
Activity of anthracycline- and ifosfamide-based chemotherapy in a series of patients affected by advanced myxofibrosarcoma. ( 28852467 )
2017
14
A histological positive margin after surgery is correlated with high local re-recurrence rate in patients with recurrent myxofibrosarcoma. ( 28064204 )
2017
15
Expanded molecular profiling of myxofibrosarcoma reveals potentially actionable targets. ( 28776571 )
2017
16
Myxofibrosarcoma of the maxillary sinus. ( 28583233 )
2017
17
Metastatic high-grade myxofibrosarcoma: review of a clinical case. ( 28730250 )
2017
18
Myxofibrosarcoma in Head and Neck: Case Report of Unusually Aggressive Presentation. ( 28893544 )
2017
19
Predictors of Local Recurrence in Patients With Myxofibrosarcoma. ( 28640064 )
2017
20
Low-Grade Myxofibrosarcoma of the Rectus Abdominus Muscle Infiltrating into Abdominal Cavity: A Case Report. ( 28293331 )
2017
21
Distant metastasis in patients with myxofibrosarcoma. ( 28814152 )
2017
22
Primary Cardiac High-grade Myxofibrosarcoma Presenting with Multiple Brain Metastases: A Case Report. ( 29375952 )
2017
23
A rare presentation of myxofibrosarcoma as a Pancoast tumor: a case report. ( 28264709 )
2017
24
Optimal Percent Myxoid Component to Predict Outcome in High-Grade Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma. ( 26759307 )
2016
25
Integrin-I+10 Dependency Identifies RAC and RICTOR as Therapeutic Targets in High-Grade Myxofibrosarcoma. ( 27577794 )
2016
26
Myxofibrosarcoma of the extremity and trunk: a multidisciplinary approach leads to good local rates of LOCAL control. ( 27909132 )
2016
27
An unexpected skin ulcer and soft tissue necrosis after the nonconcurrent combination of proton beam therapy and pazopanib: A case of myxofibrosarcoma. ( 27523716 )
2016
28
Primary cardiac myxofibrosarcoma in a young woman. ( 27594370 )
2016
29
Surgical margins do not affect prognosis in high grade myxofibrosarcoma. ( 27260849 )
2016
30
A case report of primary cardiac myxofibrosarcoma presenting with severe congestive heart failure. ( 27387491 )
2016
31
Myxofibrosarcoma presenting as an exophytic, multi-lobed nodule on the leg. ( 27617600 )
2016
32
Myxofibrosarcoma. ( 27591498 )
2016
33
Massively Distended Abdomen Caused by Fibromatosis Previously Misdiagnosed as Myxofibrosarcoma. ( 27858546 )
2016
34
Modified partial maxillary swing approach for myxofibrosarcoma in pterygopalatine fossa. ( 27501198 )
2016
35
Right cardiac intracavitary metastases from a primary intracranial myxofibrosarcoma. ( 27013654 )
2016
36
A Case of Myxofibrosarcoma in an Unusual Thoracic Location. ( 26933417 )
2016
37
Giant myxofibrosarcoma of the orbit: a rare case and a review of the literature. ( 27298287 )
2016
38
Tail of Superficial Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma After Preoperative Radiotherapy. ( 27127141 )
2016
39
Myxofibrosarcoma in the Thenar Eminence. ( 27505448 )
2016
40
Myxofibrosarcoma of the heart: A case report with positive pleural effusion cytology. ( 27546198 )
2016
41
Soft tissue myxofibrosarcoma: A clinico-pathological analysis of a series of 75 patients with emphasis on the epithelioid variant. ( 27076198 )
2016
42
Myxofibrosarcoma - Case report. ( 26982788 )
2016
43
Prognostic impact of CD109 expression in myxofibrosarcoma. ( 26031650 )
2015
44
Primary renal myxofibrosarcoma. ( 26008776 )
2015
45
Intramuscular high-grade myxofibrosarcoma of left buttock of 66-year-old male patient - approach to systematic histopathological reporting. ( 26743304 )
2015
46
Cell cycle and apoptosis regulatory proteins, proliferative markers, cell signaling molecules, CD209, and decorin immunoreactivity in low-grade myxofibrosarcoma and myxoma. ( 25940995 )
2015
47
Coronary thrombus aspiration revealed tumorous embolism of myxofibrosarcoma from the left atrium. ( 25711731 )
2015
48
High-grade myxofibrosarcoma-presented as a large mass of right upper arm. ( 25673608 )
2015
49
Primary intermediate-grade cardiac myxofibrosarcoma with osseous metaplasia: an extremely rare occurrence with a previously unreported feature. ( 25246023 )
2014
50
High-dose-rate Interstitial Brachytherapy Boost with a Pedicled Latissimus Dorsi Myocutaneous Flap for Myxofibrosarcoma of the Arm. ( 25426346 )
2014

Variations for Myxofibrosarcoma

Copy number variations for Myxofibrosarcoma from CNVD:

7 (show all 47)
# CNVD ID Chromosom 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 217848 7 104755715 105039798 Amplificationlication SRPK2 Myxofibrosarcoma
28 217923 7 105888733 105925638 Amplificationlication PBEF1 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 TIM1 Myxofibrosarcoma
35 223288 7 1855427 2272583 Amplificationlicatio n MAD1L1 Myxofibrosarcoma
36 225548 7 47314752 47621742 Amplification TNS3 Myxofibrosarcoma
37 228044 7 73245192 73247014 Amplificationlicatio n CLDN4 Myxofibrosarcoma
38 228772 7 81331444 81399452 Amplificationlicatio n HGF Myxofibrosarcoma
39 229104 7 87257728 87461612 Amplificationlicatio n RPIP9 Myxofibrosarcoma
40 229332 7 90095737 90839904 Amplificationlicatio n PFTK1 Myxofibrosarcoma
41 229600 7 92234236 92465941 Amplificationlicatio n 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 ED1 Myxofibrosarcoma
46 264546 X 67700000 72200000 Gain TED Myxofibrosarcoma
47 266520 X 98200000 110500000 Loss PLP 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:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.02 CCNA2 CREB3L1 CREB3L2
2 11.64 CCNA2 CREB3L1 CREB3L2
3
Show member pathways
11.34 CCNA2 CD36 CREB3L1 CREB3L2
4
Show member pathways
11.17 CD36 CREB3L1 CREB3L2
5 11.16 CREB3L1 CREB3L2
6 10.9 CREB3L1 CREB3L2

GO Terms for Myxofibrosarcoma

Cellular components related to Myxofibrosarcoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 nucleus GO:0005634 9.23 CCNA2 CREB3L1 CREB3L2 DES FUS MKI67

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

# Name GO ID Score Top Affiliating Genes
1 response to unfolded protein GO:0006986 8.96 CREB3L1 CREB3L2
2 endoplasmic reticulum unfolded protein response GO:0030968 8.62 CREB3L1 CREB3L2

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

# Name GO ID Score Top Affiliating Genes
1 cAMP response element binding GO:0035497 8.62 CREB3L1 CREB3L2

Sources for Myxofibrosarcoma

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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