MCID: MYX013
MIFTS: 40

Myxofibrosarcoma

Categories: Neuronal diseases, Rare diseases, Skin diseases

Aliases & Classifications for Myxofibrosarcoma

MalaCards integrated aliases for Myxofibrosarcoma:

Name: Myxofibrosarcoma 59
Fibromyxosarcoma 59 73
Dermatofibrosarcoma Protuberans, Myxoid 73
Myxoid Malignant Fibrous Histiocytoma 59

Characteristics:

Orphanet epidemiological data:

59
myxofibrosarcoma
Inheritance: Not applicable; Age of onset: Adult;

Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

Orphanet 59 ORPHA79105
UMLS via Orphanet 74 C3714524
ICD10 via Orphanet 34 C49.9

Summaries for Myxofibrosarcoma

MalaCards based summary : Myxofibrosarcoma, also known as fibromyxosarcoma, is related to malignant fibroxanthoma and embryonal sarcoma. 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 Kaposi's sarcoma-associated herpesvirus infection. The drugs Doxorubicin and Mechlorethamine have been mentioned in the context of this disorder. Affiliated tissues include heart, skin and bone.

Wikipedia : 76 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 64)
# Related Disease Score Top Affiliating Genes
1 malignant fibroxanthoma 30.1 DES MKI67 S100B
2 embryonal sarcoma 29.9 DES MKI67 S100B
3 fibrous histiocytoma 29.3 DES FUS S100B
4 dermatofibrosarcoma protuberans 29.3 DES S100B
5 malignant triton tumor 10.6 DES S100B
6 ossifying fibromyxoid tumor 10.6 DES S100B
7 syringocystadenoma papilliferum 10.6 DES S100B
8 spindle cell rhabdomyosarcoma 10.6 DES S100B
9 syringoma 10.6 DES S100B
10 juvenile xanthogranuloma 10.6 DES S100B
11 pleomorphic liposarcoma 10.6 DES S100B
12 pulmonary vein stenosis 10.6 DES MKI67
13 cutaneous fibrous histiocytoma 10.6 DES S100B
14 spindle cell sarcoma 10.5 DES S100B
15 adenosarcoma 10.5 DES MKI67
16 granular cell tumor 10.5 DES S100B
17 hemangiopericytoma, malignant 10.5 DES S100B
18 hidradenocarcinoma 10.4 MKI67 S100B
19 polymorphous low-grade adenocarcinoma 10.4 MKI67 S100B
20 odontogenic myxoma 10.4 CD36 S100B
21 neurilemmoma 10.3 DES S100B
22 pseudosarcomatous fibromatosis 10.3 CREB3L2 DES
23 atypical teratoid rhabdoid tumor 10.3 DES S100B
24 pilomatrixoma 10.3 CD36 S100B
25 babesiosis 10.2 CD36 THBS1
26 inflammatory myofibroblastic tumor 10.2 DES MKI67 S100B
27 sarcoma 10.2
28 gastrointestinal stromal tumor 10.2 DES MKI67 S100B
29 muscle cancer 10.2 CREB3L2 DES
30 ependymoma 10.1 MKI67 S100B
31 undifferentiated pleomorphic sarcoma 10.0
32 liposarcoma 10.0
33 extraskeletal ewing sarcoma 10.0 DES FUS
34 lipomatosis, multiple 9.9 DES FUS
35 histiocytoma 9.9
36 myxoid liposarcoma 9.9
37 burn scar 9.9
38 burns 9.9
39 sclerosing liposarcoma 9.8 CREB3L2 FUS
40 mixed liposarcoma 9.8 CREB3L2 FUS
41 atypical lipomatous tumor 9.8 CREB3L2 FUS
42 chondroid lipoma 9.8 CREB3L2 FUS
43 cervicitis 9.7
44 dedifferentiated liposarcoma 9.7
45 fibrosarcoma 9.7
46 cerebritis 9.7
47 neurofibrosarcoma 9.7
48 carpal tunnel syndrome 9.7
49 prostate cancer 9.7
50 cellular myxoid liposarcoma 9.7

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 14)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Doxorubicin Approved, Investigational Phase 2, Phase 3,Phase 1 23214-92-8 31703
2
Mechlorethamine Approved, Investigational Phase 2, Phase 3 51-75-2 4033
3
Ifosfamide Approved Phase 2, Phase 3 3778-73-2 3690
4
Cyclophosphamide Approved, Investigational Phase 2, Phase 3 50-18-0, 6055-19-2 2907
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 Antineoplastic Agents, Alkylating Phase 2, Phase 3
12 razoxane Phase 2
13 Protective Agents Phase 2
14 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 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
4 Sapanisertib or Pazopanib Hydrochloride in Treating Patients With Locally Advanced or Metastatic Sarcoma Recruiting NCT02601209 Phase 1, Phase 2 Pazopanib Hydrochloride;Sapanisertib
5 Doxorubicin With Upfront Dexrazoxane Plus Olaratumab for the Treatment of Advanced or Metastatic Soft Tissue Sarcoma Recruiting NCT02584309 Phase 2 Dexrazoxane;Doxorubicin;Olaratumab
6 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:

41
Heart, Skin, Bone, Lung, Brain, Ovary, Prostate

Publications for Myxofibrosarcoma

Articles related to Myxofibrosarcoma:

(show top 50) (show all 205)
# Title Authors Year
1
Myxofibrosarcoma of unusual sites. ( 29065228 )
2018
2
Left atrial appendage myxofibrosarcoma: A rare masquerader of myxoma and thrombus-"all that glitters is not gold". ( 29280521 )
2018
3
Primary cardiac myxofibrosarcoma: case report, literature review and pooled analysis. ( 29720127 )
2018
4
Biology and Management of Undifferentiated Pleomorphic Sarcoma, Myxofibrosarcoma, and Malignant Peripheral Nerve Sheath Tumors: State of the Art and Perspectives. ( 29220302 )
2018
5
Primary myxofibrosarcoma of the kidney. ( 29075931 )
2018
6
An entire clinical course of mediastinal myxofibrosarcoma treated by surgery and radiation. ( 29748938 )
2018
7
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
8
Pembrolizumab for Refractory Metastatic Myxofibrosarcoma: A Case Report. ( 29361819 )
2018
9
A rare presentation of myxofibrosarcoma as a Pancoast tumor: a case report. ( 28264709 )
2017
10
Myxofibrosarcoma of the maxillary sinus. ( 28583233 )
2017
11
Metastatic high-grade myxofibrosarcoma: review of a clinical case. ( 28730250 )
2017
12
Low-Grade Myxofibrosarcoma of the Rectus Abdominus Muscle Infiltrating into Abdominal Cavity: A Case Report. ( 28293331 )
2017
13
Primary Cardiac High-grade Myxofibrosarcoma Presenting with Multiple Brain Metastases: A Case Report. ( 29375952 )
2017
14
High-grade myxofibrosarcoma of the abdominal wall. ( 28576911 )
2017
15
Myxofibrosarcoma in Head and Neck: Case Report of Unusually Aggressive Presentation. ( 28893544 )
2017
16
Predictors of Local Recurrence in Patients With Myxofibrosarcoma. ( 28640064 )
2017
17
Low grade myxofibrosarcoma in the right ventricle presenting as pulmonary thromboembolism. ( 29312771 )
2017
18
A histological positive margin after surgery is correlated with high local re-recurrence rate in patients with recurrent myxofibrosarcoma. ( 28064204 )
2017
19
Myxofibrosarcoma primary cultures: molecular and pharmacological profile. ( 29449896 )
2017
20
Expanded molecular profiling of myxofibrosarcoma reveals potentially actionable targets. ( 28776571 )
2017
21
Myxofibrosarcoma: First report of myxofibrosarcoma of bone arising at a bone infarct. ( 28429048 )
2017
22
Discoidin, CUB and LCCL domain-containing protein 2 (DCBLD2) is a novel biomarker of myxofibrosarcoma invasion identified by global protein expression profiling. ( 28668639 )
2017
23
Establishment of a novel cellular model for myxofibrosarcoma heterogeneity. ( 28304377 )
2017
24
Distant metastasis in patients with myxofibrosarcoma. ( 28814152 )
2017
25
Claudin 6 expression is useful to distinguish myxofibrosarcomas from other myxoid soft tissue tumors. ( 28476380 )
2017
26
Activity of anthracycline- and ifosfamide-based chemotherapy in a series of patients affected by advanced myxofibrosarcoma. ( 28852467 )
2017
27
A Case of Myxofibrosarcoma in an Unusual Thoracic Location. ( 26933417 )
2016
28
Myxofibrosarcoma of the extremity and trunk: a multidisciplinary approach leads to good local rates of LOCAL control. ( 27909132 )
2016
29
Myxofibrosarcoma presenting as an exophytic, multi-lobed nodule on the leg. ( 27617600 )
2016
30
A case report of primary cardiac myxofibrosarcoma presenting with severe congestive heart failure. ( 27387491 )
2016
31
Primary cardiac myxofibrosarcoma in a young woman. ( 27594370 )
2016
32
Massively Distended Abdomen Caused by Fibromatosis Previously Misdiagnosed as Myxofibrosarcoma. ( 27858546 )
2016
33
An unexpected skin ulcer and soft tissue necrosis after the nonconcurrent combination of proton beam therapy and pazopanib: A case of myxofibrosarcoma. ( 27523716 )
2016
34
Myxofibrosarcoma in the Thenar Eminence. ( 27505448 )
2016
35
Soft tissue myxofibrosarcoma: A clinico-pathological analysis of a series of 75 patients with emphasis on the epithelioid variant. ( 27076198 )
2016
36
Giant myxofibrosarcoma of the orbit: a rare case and a review of the literature. ( 27298287 )
2016
37
Modified partial maxillary swing approach for myxofibrosarcoma in pterygopalatine fossa. ( 27501198 )
2016
38
Tail of Superficial Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma After Preoperative Radiotherapy. ( 27127141 )
2016
39
Integrin-I+10 Dependency Identifies RAC and RICTOR as Therapeutic Targets in High-Grade Myxofibrosarcoma. ( 27577794 )
2016
40
Myxofibrosarcoma of the heart: A case report with positive pleural effusion cytology. ( 27546198 )
2016
41
Surgical margins do not affect prognosis in high grade myxofibrosarcoma. ( 27260849 )
2016
42
Myxofibrosarcoma. ( 27591498 )
2016
43
Optimal Percent Myxoid Component to Predict Outcome in High-Grade Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma. ( 26759307 )
2016
44
Right cardiac intracavitary metastases from a primary intracranial myxofibrosarcoma. ( 27013654 )
2016
45
Myxofibrosarcoma - Case report. ( 26982788 )
2016
46
Coronary thrombus aspiration revealed tumorous embolism of myxofibrosarcoma from the left atrium. ( 25711731 )
2015
47
Primary renal myxofibrosarcoma. ( 26008776 )
2015
48
Intramuscular high-grade myxofibrosarcoma of left buttock of 66-year-old male patient - approach to systematic histopathological reporting. ( 26743304 )
2015
49
High-grade myxofibrosarcoma-presented as a large mass of right upper arm. ( 25673608 )
2015
50
Cell cycle and apoptosis regulatory proteins, proliferative markers, cell signaling molecules, CD209, and decorin immunoreactivity in low-grade myxofibrosarcoma and myxoma. ( 25940995 )
2015

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

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 9.37 CREB3L1 CREB3L2
2 negative regulation of sprouting angiogenesis GO:1903671 9.32 CREB3L1 THBS1
3 positive regulation of reactive oxygen species metabolic process GO:2000379 9.26 CD36 THBS1
4 negative regulation of fibroblast growth factor receptor signaling pathway GO:0040037 9.16 CREB3L1 THBS1
5 positive regulation of blood coagulation GO:0030194 8.96 CD36 THBS1
6 response to unfolded protein GO:0006986 8.8 CREB3L1 CREB3L2 THBS1

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

# Name GO ID Score Top Affiliating Genes
1 transforming growth factor beta binding GO:0050431 9.16 CD36 THBS1
2 low-density lipoprotein particle binding GO:0030169 8.96 CD36 THBS1
3 cAMP response element binding GO:0035497 8.62 CREB3L1 CREB3L2

Sources for Myxofibrosarcoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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