MCID: DRM004
MIFTS: 52

Dermatofibrosarcoma malady

Cancer diseases, Skin diseases categories
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Summaries for Dermatofibrosarcoma

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65Wikipedia, 33MalaCards
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Wikipedia:65 Dermatofibrosarcoma protuberans (DFSP) is a very rare tumor. It is a rare neoplasm of the dermis layer... more...

MalaCards: Dermatofibrosarcoma, also known as dermatofibrosarcoma nos, is related to dermatofibrosarcoma protuberans and fibrous histiocytoma. An important gene associated with Dermatofibrosarcoma is PDGFB (platelet-derived growth factor beta polypeptide), and among its related pathways are Response to elevated platelet cytosolic Ca2+ and G protein signaling H RAS regulation pathway. The compounds dmpq dihydrochloride and su 16f have been mentioned in the context of this disorder. Affiliated tissues include skin, breast and lung, and related mouse phenotypes are reproductive system and integument.

Aliases & Classifications for Dermatofibrosarcoma

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8Disease Ontology, 10DISEASES, 45Novoseek, 62UMLS, 58SNOMED-CT, 35MeSH
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer diseases
Anatomical: Skin diseases


Aliases & Descriptions:

dermatofibrosarcoma 8 10 45 62
dermatofibrosarcoma nos 8


External Ids:

Disease Ontology8 DOID:3507

Related Diseases for Dermatofibrosarcoma

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17GeneCards, 18GeneDecks
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Diseases related to Dermatofibrosarcoma via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 117)
idRelated DiseaseScoreTop Affiliating Genes
1dermatofibrosarcoma protuberans31.6S100B, PDGFRB, COL1A1, ACTC1, F13A1, DES
2fibrous histiocytoma30.3ACTC1, DES, S100B, F13A1
3fibroma30.3ACTC1, F13A1, DES
4fibrosarcoma30.3PDGFRB, ACTC1, DES
5sarcoma30.2DES, F13A1, ACTC1, PDGFRB, PDGFB, S100B
6papilloma30.0ACTC1
7kaposi's sarcoma30.0F13A1, PDGFB
8glomus tumor30.0ACTC1, DES
9basal cell carcinoma29.9ACTC1, F13A1
10melanoma29.8F13A1, ACTC1, PDGFRB, PDGFB, S100B, DES
11meningioma29.8PDGFB, PDGFRB, S100B
12adenocarcinoma29.8PDGFRB, ACTC1, F13A1, PDGFB
13giant cell tumor29.8F13A1, S100B, DES, ACTC1
14gastrointestinal stromal tumor29.7PDGFRB, ACTC1, DES, S100B
15histiocytoma10.3
16infantile myofibromatosis10.1DES
17fasciitis10.1ACTC1
18burn scar10.1
19burns10.1
20spindle cell lipoma10.1
21merkel cell carcinoma10.1
22lipoma10.1
23pancreatitis10.1
24bednar's tumor10.1
25myxofibrosarcoma10.1
26fibrous meningioma10.1S100B
27gingival overgrowth10.1PDGFB, COL1A1
28syringoma10.1S100B
29perineurioma10.1S100B
30ganglioglioma10.1S100B
31epithelioid leiomyosarcoma10.0ACTC1, DES
32pseudosarcomatous fibromatosis10.0DES, ACTC1
33spindle cell carcinoma10.0ACTC1, DES
34adenosarcoma10.0DES, ACTC1
35rhabdoid tumor10.0ACTC1, DES
36renal fibrosis10.0PDGFB, PDGFRB
37angiomyolipoma10.0ACTC1, DES
38embryonal rhabdomyosarcoma10.0ACTC1, DES
39hemangioma10.0ACTC1, F13A1
40abrikosov's tumor10.0DES, ACTC1
41uterine sarcoma10.0PDGFRB, DES
42idiopathic pulmonary fibrosis10.0PDGFB, ACTC1
43esophageal cancer10.0PDGFRB, PDGFB
44juvenile xanthogranuloma10.0S100B, F13A1
45histiocytosis10.0S100B, F13A1
46malignant mesothelioma10.0PDGFRB, DES
47granular cell tumor10.0S100B, DES
48myoepithelioma10.0S100B, ACTC1
49pulmonary fibrosis10.0PDGFRB, COL1A1, PDGFB
50sex cord-gonadal stromal tumor10.0S100B, DES

Graphical network of the top 20 diseases related to Dermatofibrosarcoma:



Diseases related to dermatofibrosarcoma

Symptoms for Dermatofibrosarcoma

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Drugs & Therapeutics for Dermatofibrosarcoma

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Sources:
42NIH Clinical Center, 6ClinicalTrials, 62UMLS, 41NDF-RT
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Drug clinical trials:

Search ClinicalTrials for Dermatofibrosarcoma

Search NIH Clinical Center for Dermatofibrosarcoma

Genetic Tests for Dermatofibrosarcoma

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Anatomical Context for Dermatofibrosarcoma

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33MalaCards
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MalaCards organs/tissues related to Dermatofibrosarcoma:

33
Skin, Breast, Lung, Bone, Lymph node, Thyroid, Endothelial

Animal Models for Dermatofibrosarcoma or affiliated genes

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37MGI
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MGI Mouse Phenotypes related to Dermatofibrosarcoma:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00053898.6F13A1, COL1A1, PDGFRB, PDGFB
2MP:00107718.1S100B, PDGFB, PDGFRB, COL1A1
3MP:00053698.1DES, PDGFB, ACTC1, PDGFRB, COL1A1
4MP:00028737.8S100B, PDGFRB, COL1A1, ACTC1
5MP:00053857.7ACTC1, PDGFRB, COL1A1, F13A1, DES, PDGFB
6MP:00053767.6PDGFRB, DES, F13A1, ACTC1, COL1A1, PDGFB
7MP:00107687.6PDGFB, PDGFRB, COL1A1, ACTC1, F13A1, DES

Publications for Dermatofibrosarcoma

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52PubMed
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Articles related to Dermatofibrosarcoma:

(show top 50)    (show all 530)
idTitleAuthorsYear
1
Outcome of wide local excision in dermatofibrosarcoma protuberans and use of radiotherapy for margin-positive disease. (23741663)
2013
2
Dermatofibrosarcoma protuberans in a 10-year-old child. (24421865)
2013
3
Painful late recurrence of dermatofibrosarcoma protuberans of breast in a centurian female. (24298499)
2013
4
Abdominoplasty and thoraco-epigastric flaps for large anterior trunk defects after dermatofibrosarcoma protuberans wide resection: Two illustrative cases. (23219975)
2013
5
CD99 Expression in Dermatofibrosarcoma Protuberans and Dermatofibroma. (24247571)
2013
6
Magnetic resonance imaging of vulvar dermatofibrosarcoma protuberans - report of a case. (24133389)
2013
7
Practice gaps. Underuse of Mohs micrographic surgery for the treatment of dermatofibrosarcoma protuberans: comment on "efficacy of mohs micrographic surgery for the treatment of dermatofibrosarcoma protuberans". (22986860)
2012
8
Dermatofibrosarcoma protuberans: Our experience of 59 cases. (23162649)
2012
9
Dermatofibrosarcoma: a rare neoplasm of skin. (23175016)
2012
10
Dermatofibrosarcoma protuberans in childhood. (23154247)
2012
11
Treatment of advanced dermatofibrosarcoma protuberans with imatinib mesylate with or without surgical resection. (20569296)
2011
12
Molecular diagnosis of dermatofibrosarcoma protuberans: a comparison between reverse transcriptase-polymerase chain reaction and fluorescence in situ hybridization methodologies. (21484928)
2011
13
Recurrent bilateral dermatofibrosarcoma protuberans of eyelids. (21346665)
2011
14
A case of childhood dermatofibrosarcoma protuberans of the face. (21763849)
2011
15
Dermatofibrosarcoma protuberans with areas of giant cell fibroblastoma in the vulva: a case report. (21319518)
2010
16
A novel COL1A1 exon 14/PDGFB fusion gene in dermatofibrosarcoma protuberans. (20133221)
2010
17
Imaging of dermatofibrosarcoma protuberans of breast. (20626395)
2010
18
Imatinib mesylate as a preoperative therapy in dermatofibrosarcoma: results of a multicenter phase II study on 25 patients. (20439456)
2010
19
Imatinib mesylate in advanced dermatofibrosarcoma protuberans: pooled analysis of two phase II clinical trials. (20194851)
2010
20
Cutaneous CD34+ spindle cell neoplasms: Histopathologic features distinguish spindle cell lipoma, solitary fibrous tumor, and dermatofibrosarcoma protuberans. (20559119)
2010
21
Medallion-like dermal dendrocyte hamartoma: the main diagnostic pitfall is congenital atrophic dermatofibrosarcoma. (19016705)
2009
22
Surgical treatment for dermatofibrosarcoma protuberans: the Dartmouth experience and literature review. (18443511)
2008
23
Short German guidelines: dermatofibrosarcoma protuberans. (18801137)
2008
24
Atrophic dermatofibrosarcoma protuberans with the fusion gene COL1A1-PDGFB. (18005119)
2008
25
Mohs micrographic surgery and dermatofibrosarcoma protuberans: a multidisciplinary approach in 44 patients. (18520205)
2008
26
Myxoid dermatofibrosarcoma protuberans: a rare variant analyzed in a series of 23 cases. (17721193)
2007
27
Dermatofibrosarcoma protuberans and small bowel adenocarcinoma: coincidental occurrence or genetic association? (18175227)
2006
28
Dermatofibrosarcoma protuberans: report of a case with a variant ring chromosome and metastases following pregnancy. (16640548)
2006
29
Dermatofibrosarcoma protuberans arising in a decorative tattoo. (18521416)
2005
30
Dermatofibrosarcoma protuberans occuring in a child. (15943042)
2005
31
Molecular and clinical analysis of locally advanced dermatofibrosarcoma protuberans treated with imatinib: Imatinib Target Exploration Consortium Study B2225. (15681532)
2005
32
Molecularly targeted treatment for dermatofibrosarcoma protuberans. (15176002)
2004
33
Dermatofibrosarcoma protuberans: a report on 29 patients treated by Mohs micrographic surgery with long-term follow-up and review of the literature. (15221986)
2004
34
Neurofibromatous changes in dermatofibrosarcoma protuberans: a potential pitfall in the diagnosis of a serious cutaneous soft tissue neoplasm. (15239679)
2004
35
Dermatofibrosarcoma protuberans arising from a burn scar of the axilla. (15084891)
2004
36
Dermatofibrosarcoma protuberans. (15503305)
2004
37
Dermatofibrosarcoma protruberans in a renal transplant recipient. (12558754)
2003
38
Giant fibrosarcoma arising in dermatofibrosarcoma protuberans on the scalp during pregnancy. (12614428)
2003
39
Dermatofibrosarcoma protuberans of the vulva and groin: detection of COL1A1-PDGFB fusion transcripts by RT-PCR. (12641779)
2003
40
COL1A1-PDGFB gene fusion demonstrates a common histogenetic origin for dermatofibrosarcoma protuberans and its granular cell variant. (12131162)
2002
41
Composite dermatofibrosarcoma protuberans-giant cell fibroblastoma recurring as BednA!r tumor-giant cell fibroblastoma with mucoid lakes and with amputation neuroma. (12629865)
2002
42
Dermatofibrosarcoma protuberans: experience with 14 cases. (11763383)
2001
43
Dermatofibrosarcoma protuberans: A clinicopathologic analysis of patients treated and followed at a single institution. (10870053)
2000
44
A morphologic study of dermatofibrosarcoma protuberans: expansion of a histologic profile. (10774935)
2000
45
Dermatofibrosarcoma protuberans of the head and neck. (11034249)
2000
46
Dermatofibrosarcoma protuberans: what is the best surgical approach? (11128831)
1999
47
Darier-White disease and dermatofibrosarcoma protuberans. (10592418)
1999
48
Acral dermatofibrosarcoma protuberans with metastases. (10029491)
1999
49
A cutaneous case of giant cell angiofibroma occurring with dermatofibrosarcoma protuberans and showing bimodal CD34+ fibroblastic and FXIIIa+ histiocytic immunophenotype. (9696292)
1998
50
Sclerosing dermatofibrosarcoma protuberans. (9826170)
1998

Variations for Dermatofibrosarcoma

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Expression for genes affiliated with Dermatofibrosarcoma

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2BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Dermatofibrosarcoma

Search GEO for disease gene expression data for Dermatofibrosarcoma.

Pathways for genes affiliated with Dermatofibrosarcoma

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50PathCards, 55Reactome, 60Thomson Reuters, 38NCBI BioSystems Database, 53QIAGEN, 30KEGG
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Pathways related to Dermatofibrosarcoma according to GeneCards/GeneDecks:

(show all 13)
idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
9.6F13A1, PDGFB
29.5PDGFB, PDGFRB
3
Show member pathways
PDGF Pathway38
9.5PDGFB, PDGFRB
49.5PDGFB, PDGFRB
59.5PDGFB, PDGFRB
69.5PDGFB, PDGFRB
7
Show member pathways
9.4DES, ACTC1
8
Show member pathways
9.1COL1A1, PDGFB, PDGFRB
9
Show member pathways
Integrin-mediated cell adhesion38
Focal Adhesion38
9.1PDGFRB, COL1A1, PDGFB
109.1PDGFB, PDGFRB, COL1A1
119.1PDGFB, PDGFRB, COL1A1
12
Show member pathways
8.7S100B, PDGFRB, PDGFB
13
Show member pathways
8.5DES, COL1A1, PDGFRB, PDGFB

Compounds for genes affiliated with Dermatofibrosarcoma

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61Tocris Bioscience, 45Novoseek, 11DrugBank, 29IUPHAR, 3BitterDB, 51PharmGKB, 24HMDB
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Compounds related to Dermatofibrosarcoma according to GeneCards/GeneDecks:

(show all 44)
idCompoundScoreTop Affiliating Genes
1dmpq dihydrochloride619.8PDGFRB, PDGFB
2su 16f619.8PDGFRB, PDGFB
3su 6668619.8PDGFRB, PDGFB
4sunitinib malate619.8PDGFRB, PDGFB
5bleomycin45 1110.7PDGFB, COL1A1
6ecori459.5COL1A1, S100B
7silver459.5PDGFB, F13A1, DES
83-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide459.3S100B, DES
9ccl445 2910.2DES, S100B
10chloramphenicol45 3 51 1112.1S100B, PDGFB, COL1A1
11ganglioside459.0PDGFRB, DES
12orcein459.0S100B, ACTC1, DES
13mucicarmine459.0S100B, ACTC1, DES
14ribonucleic acid458.9ACTC1, COL1A1, PDGFRB, PDGFB
15formaldehyde45 249.9S100B, ACTC1, DES
16oligonucleotide458.9F13A1, COL1A1, PDGFRB, PDGFB
17cyclosporin a45 29 6110.9S100B, PDGFB, ACTC1
18glycogen45 249.8DES, ACTC1, S100B
19methionine458.7S100B, PDGFRB, DES
20thymidine45 249.7DES, COL1A1, PDGFB, S100B
21proline458.7DES, PDGFRB, S100B
22dexamethasone45 51 29 1111.6ACTC1, COL1A1, PDGFB, S100B
23procollagen458.6DES, F13A1, ACTC1, COL1A1, PDGFB
24creatinine458.6DES, ACTC1, S100B
25s 100458.6DES, F13A1, ACTC1, S100B
26reticulin458.6S100B, ACTC1, F13A1, DES
27heparin45 29 24 1111.6S100B, F13A1, DES
28b 723458.6DES, F13A1, ACTC1, S100B
29hematoxylin458.6DES, F13A1, ACTC1, S100B
30bromodeoxyuridine458.5DES, PDGFRB, PDGFB, S100B
31iron45 249.5DES, F13A1, ACTC1, S100B
32cysteine458.3S100B, PDGFRB, COL1A1, DES
33testosterone45 61 24 1111.3S100B, PDGFRB, ACTC1, DES
34serine458.0DES, COL1A1, PDGFRB, PDGFB, S100B
35oxygen45 249.0S100B, PDGFB, PDGFRB, ACTC1, DES
36glucose458.0DES, ACTC1, PDGFRB, PDGFB, S100B
37retinoic acid45 249.0DES, ACTC1, COL1A1, PDGFRB, S100B
38tyrosine457.9DES, ACTC1, PDGFRB, PDGFB, S100B
39imatinib45 51 119.6S100B, PDGFB, PDGFRB, COL1A1, ACTC1, DES
40paraffin457.6DES, F13A1, ACTC1, PDGFRB, PDGFB, S100B
41progesterone45 29 61 24 1111.6DES, F13A1, ACTC1, PDGFRB, PDGFB, S100B
42calcium45 51 24 1110.5DES, F13A1, ACTC1, COL1A1, PDGFRB, S100B
43vegf457.2DES, F13A1, ACTC1, COL1A1, PDGFRB, PDGFB
44estrogen457.2DES, F13A1, ACTC1, COL1A1, PDGFRB, PDGFB

GO Terms for genes affiliated with Dermatofibrosarcoma

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16Gene Ontology
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Cellular components related to Dermatofibrosarcoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1platelet alpha granule lumenGO:0310939.3F13A1, PDGFB
2extracellular regionGO:0055768.4S100B, PDGFB, COL1A1, F13A1
3extracellular spaceGO:0056158.2S100B, PDGFB, COL1A1, ACTC1

Biological processes related to Dermatofibrosarcoma according to GeneCards/GeneDecks:

(show all 26)
idNameGO IDScoreTop Affiliating Genes
1metanephric glomerular mesangial cell proliferation involved in metanephros developmentGO:0722629.9PDGFB, PDGFRB
2positive regulation of metanephric mesenchymal cell migration by platelet-derived growth factor receptor-beta signaling pathwayGO:0357939.8PDGFB, PDGFRB
3positive regulation of DNA biosynthetic processGO:20005739.8PDGFRB, PDGFB
4positive regulation of calcium ion importGO:0902809.8PDGFRB, PDGFB
5positive regulation of chemotaxisGO:0509219.8PDGFRB, PDGFB
6positive regulation of smooth muscle cell migrationGO:0149119.8PDGFB, PDGFRB
7positive regulation of phosphatidylinositol 3-kinase activityGO:0435529.8PDGFB, PDGFRB
8platelet-derived growth factor receptor signaling pathwayGO:0480089.8PDGFB, PDGFRB
9positive regulation of reactive oxygen species metabolic processGO:20003799.8PDGFB, PDGFRB
10positive regulation of mitosisGO:0458409.7PDGFRB, PDGFB
11positive regulation of smooth muscle cell proliferationGO:0486619.7PDGFB, PDGFRB
12muscle filament slidingGO:0300499.7ACTC1, DES
13response to hydrogen peroxideGO:0425429.7PDGFRB, COL1A1
14cell chemotaxisGO:0603269.7PDGFRB, PDGFB
15cardiac myofibril assemblyGO:0550039.7ACTC1, PDGFRB
16positive regulation of MAP kinase activityGO:0434069.7PDGFB, PDGFRB
17platelet degranulationGO:0025769.6PDGFB, F13A1
18positive regulation of phosphatidylinositol 3-kinase signalingGO:0140689.6PDGFB, PDGFRB
19platelet activationGO:0301689.5F13A1, COL1A1, PDGFB
20positive regulation of ERK1 and ERK2 cascadeGO:0703749.5PDGFB, PDGFRB
21response to estradiolGO:0323559.4PDGFB, PDGFRB, COL1A1
22positive regulation of cell migrationGO:0303359.4PDGFB, PDGFRB, COL1A1
23peptidyl-tyrosine phosphorylationGO:0181089.4PDGFRB, PDGFB
24blood coagulationGO:0075969.3PDGFB, COL1A1, F13A1
25positive regulation of cell proliferationGO:0082848.8PDGFRB, PDGFB, S100B
26innate immune responseGO:0450878.4S100B, PDGFB, PDGFRB

Molecular functions related to Dermatofibrosarcoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1platelet-derived growth factor receptor bindingGO:0051619.5PDGFB, PDGFRB
2platelet-derived growth factor bindingGO:0484079.0PDGFB, PDGFRB, COL1A1
3identical protein bindingGO:0428028.0S100B, PDGFB, COL1A1, DES

Products for genes affiliated with Dermatofibrosarcoma

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Sources for Dermatofibrosarcoma

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4CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet