MCID: DRM004
MIFTS: 53

Dermatofibrosarcoma malady

Summaries for Dermatofibrosarcoma

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63Wikipedia, 32MalaCards
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Wikipedia:63 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 sarcoma. An important gene associated with Dermatofibrosarcoma is PDGFB (platelet-derived growth factor beta polypeptide), and among its related pathways are Osteoblast Signaling and Striated Muscle Contraction. The compounds estrogen and procollagen have been mentioned in the context of this disorder. Affiliated tissues include skin, breast and lung, and related mouse phenotypes are muscle and integument.

Aliases & Classifications for Dermatofibrosarcoma

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8Disease Ontology, 10DISEASES, 44Novoseek, 60UMLS, 34MeSH, 56SNOMED-CT
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Aliases & Descriptions:

dermatofibrosarcoma 8 10 44 60
dermatofibrosarcoma nos 8


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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 186)
idRelated DiseaseScoreTop Affiliating Genes
1dermatofibrosarcoma protuberans30.8S100A1, PDGFB, PDGFRB, F13A1, DES, MUC1
2sarcoma30.4S100B, VIM, S100A1, PDGFB, PDGFRB, ACTC1
3fibrous histiocytoma30.4CD34
4fibrosarcoma30.3VIM, PDGFRB, NES, DES, CD34, ACTC1
5giant cell tumor30.3VIM, S100B, CTSK, F13A1, DES, MUC1
6lipoma30.0CD34, DES, S100A1
7melanoma30.0DES, CD34, EIF2AK3, MMP11, KIT, ACTC1
8papilloma29.8ACTC1
9cutaneous fibrous histiocytoma29.8MMP11, CD34
10endometriosis29.8ACTC1, MMP11
11epithelioid sarcoma29.8VIM, MUC1, CD34
12choroid plexus papilloma29.8MUC1, S100B
13kaposi's sarcoma29.8PDGFB, F13A1, CD34
14glomus tumor29.8ACTC1, DES, S100A1, VIM
15basal cell carcinoma29.8F13A1, CD34, MMP11, ACTC1
16spindle cell sarcoma29.8VIM, S100B, DES, MUC1, ACTC1
17meningioma29.8VIM, S100B, S100A1, PDGFB, PDGFRB, MUC1
18breast cancer29.8VIM, S100A1, APOD, MUC1, MMP11, ACTC1
19gastrointestinal stromal tumor29.8VIM, S100B, S100A1, PDGFRB, NES, DES
20adenocarcinoma29.8VIM, PDGFB, PDGFRB, NES, F13A1, MUC1
21histiocytoma10.3
22burn scar10.0
23burns10.0
24merkel cell carcinoma10.0
25spindle cell lipoma10.0
26pancreatitis10.0
27skin sarcoma10.0
28bednar's tumor10.0
29hepatitis10.0PDGFB
30glomerulonephritis10.0PDGFB
31lymphangioleiomyomatosis10.0DES
32gliosarcoma10.0PDGFRB
33fasciitis10.0ACTC1
34leydig cell tumor10.0PDGFRB
35uterine carcinosarcoma10.0KIT
36infantile myofibromatosis10.0DES
37renal oncocytoma10.0S100A1
38dermatitis10.0F13A1
39malignant fibroxanthoma10.0CD34
40neurilemmoma10.0CD34
41stomach cancer10.0CD34
42carney triad10.0KIT
43hemangioendothelioma10.0CD34
44cholangiocarcinoma10.0MUC1
45liver cirrhosis10.0PDGFB
46adenomatoid tumor10.0VIM
47sertoli-leydig cell tumor10.0MUC1
48histiocytosis10.0CD163, S100B
49endometrial carcinoma10.0CD34, APOD
50pneumonia10.0MUC1

Graphical network of the top 20 diseases related to Dermatofibrosarcoma:



Diseases related to dermatofibrosarcoma

Clinical Features for Dermatofibrosarcoma

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Drugs & Therapeutics for Dermatofibrosarcoma

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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Dermatofibrosarcoma

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Anatomical Context for Dermatofibrosarcoma

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32MalaCards
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MalaCards organs/tissues related to Dermatofibrosarcoma:

32
Skin, Breast, Lung, Bone, Lymph node, Thyroid, Endothelial

Animal Models for Dermatofibrosarcoma or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Dermatofibrosarcoma:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:000536910.3PDGFB, PDGFRB, ACTC1
2MP:001077110.0CD34, PDGFB, S100B, COL1A1
3MP:00053799.7PDGFRB, CTSK, EIF2AK3, KIT
4MP:00053859.5COL1A1, VIM, S100A1, PDGFB, PDGFRB, F13A1
5MP:00053869.3COL1A1, VIM, S100B, S100A1, APOD, NES
6MP:00107689.3COL1A1, VIM, PDGFB, PDGFRB, APOD, NES
7MP:00053769.0ACTC1, COL1A1, VIM, PDGFB, PDGFRB, APOD

Publications for Dermatofibrosarcoma

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50PubMed
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Articles related to Dermatofibrosarcoma:

(show top 50)    (show all 537)
idTitleAuthorsYear
1
Outcome of wide local excision in dermatofibrosarcoma protuberans and use of radiotherapy for margin-positive disease. (23741663)
2013
2
Magnetic resonance imaging of vulvar dermatofibrosarcoma protuberans - report of a case. (24133389)
2013
3
Giant Dermatofibrosarcoma Protuberans of the Anterior Chest Wall. (24388271)
2013
4
Positive margins in excised dermatofibrosarcoma protuberans: a study of 58 cases treated with slow-Mohs surgery. (23931335)
2013
5
High immunohistochemical nestin expression is associated with greater depth of infiltration in dermatofibrosarcoma protuberans: a study of 71 cases. (23962157)
2013
6
Familial dermatofibrosarcoma protuberans: a nonfortuitous association? (23627813)
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
A rapid and efficient newly established method to detect COL1A1-PDGFB gene fusion in dermatofibrosarcoma protuberans. (22842571)
2012
9
Dermatofibrosarcoma protuberans: dealing with slow Mohs procedures employing formalin-fixed, paraffin wax-embedded tissue in a busy diagnostic laboratory. (22872928)
2012
10
Dermatofibrosarcoma protuberans: clinical diagnoses and treatment results of 260 cases in China. (21815146)
2012
11
A case of childhood dermatofibrosarcoma protuberans of the face. (21763849)
2011
12
Dermatofibrosarcoma: a rare form of soft tissue. Management and review of the literature. (22165066)
2011
13
Simultaneous diagnosis of mycosis fungoides and dermatofibrosarcoma protuberans in a young patient: is it just a coincidence? (21781083)
2011
14
Plaque-like CD34-positive dermal fibroma ("medallion-like dermal dendrocyte hamartoma"): clinicopathologic, immunohistochemical, and molecular analysis of 5 cases emphasizing its distinction from superficial, plaque-like dermatofibrosarcoma protuberans. (20061935)
2010
15
Current treatment options in dermatofibrosarcoma protuberans. (19205737)
2009
16
Merkel cell carcinoma metastasis and dermatofibrosarcoma protuberans presenting as a collision tumour: a case report and review of the literature. (19830210)
2009
17
Expression of nestin in dermatofibrosarcoma protuberans in comparison to dermatofibroma. (18705829)
2008
18
Dermatofibrosarcoma protuberans: a case report and review of the literature. (21103308)
2008
19
Peripheral excision margins for dermatofibrosarcoma protuberans. (18574636)
2008
20
Re-evaluation of CD34-negative dermatofibrosarcoma protuberans with a highly sensitive, tyramide-based immunohistochemical analysis. (19049941)
2008
21
Myxoid dermatofibrosarcoma protuberans: a rare variant analyzed in a series of 23 cases. (17721193)
2007
22
Microscopic margins and results of surgery for dermatofibrosarcoma protuberans. (17440355)
2007
23
Dermatofibrosarcoma protuberans. (17509257)
2007
24
Excellent survival of pediatric dermatofibrosarcoma protuberans in Taiwanese. (17109179)
2007
25
Intracranial recurrence of the scalp dermatofibrosarcoma. (16757109)
2007
26
Imatinib mesylate in a patient with metastatic disease originating from a dermatofibrosarcoma protuberans of the scalp. (17075323)
2006
27
Imatinib mesylate in the treatment of a large unresectable dermatofibrosarcoma protuberans: a case study. (16918576)
2006
28
Mohs micrographic surgery and partial amputation preserving function and aesthetics in digits: case reports of invasive melanoma and digital dermatofibrosarcoma protuberans. (17199665)
2006
29
Overexpression of phosphorylated-STAT3 and phosphorylated-ERK protein in dermatofibrosarcoma protuberans. (16709490)
2006
30
Dermatofibrosarcoma protuberans: review of 20-years experience. (16952850)
2006
31
Fine needle aspiration cytology of dermatofibrosarcoma protuberans in the breast: a case report. (15875741)
2005
32
Dermatofibrosarcoma protuberans following tanning bed use. (16302562)
2005
33
Gene mutation analysis in five cases of dermatofibrosarcoma protuberans using formalin-fixed, paraffin-embedded tissues. (16040406)
2005
34
Neurofibromatous changes in dermatofibrosarcoma protuberans: a potential pitfall in the diagnosis of a serious cutaneous soft tissue neoplasm. (15239679)
2004
35
Analysis of gene mutations in three cases of dermatofibrosarcoma protuberans (DFSP): ordinary DFSP, DFSP with fibrosarcomatous lesion (DFSP-FS) and lung metastasis of DFSP-FS. (14643521)
2003
36
Differential sensitivity to imatinib of 2 patients with metastatic sarcoma arising from dermatofibrosarcoma protuberans. (12209598)
2002
37
Dermatofibrosarcoma protuberans with lung metastasis in a patient with progressive systemic sclerosis. (11902708)
2002
38
Tenascin differentiates dermatofibroma from dermatofibrosarcoma protuberans: comparison with CD34 and factor XIIIa. (11172295)
2001
39
Myoid cells in the fibrosarcomatous variant of dermatofibrosarcoma protuberans. (10733324)
2000
40
Palisading and verocay body-prominent dermatofibrosarcoma protuberans: a report of three cases. (11119127)
2000
41
Dermatofibrosarcoma protuberans growing around plantar aponeurosis: excision by Mohs micrographic surgery. (11050499)
2000
42
Darier-White disease and dermatofibrosarcoma protuberans. (10592418)
1999
43
Detection of COL1A1-PDGFB fusion transcripts in dermatofibrosarcoma protuberans by reverse transcription-polymerase chain reaction using archival formalin-fixed, paraffin-embedded tissues. (10565681)
1999
44
Giant cell fibroblastoma: a variant of dermatofibrosarcoma protruberans treated with Mohs' micrographic surgery. (10469104)
1999
45
The atrophic variant of dermatofibrosarcoma protuberans in childhood: a report of six cases. (10025975)
1998
46
A case of recurrent dermatofibrosarcoma protuberans. (9713472)
1998
47
A case of dermatofibrosarcoma protuberans with a ring chromosome 5 and a rearranged chromosome 22 containing amplified COL1A1 and PDGFB sequences. (10072161)
1998
48
Expression of the human hematopoietic progenitor cell antigen CD34 in dermatofibrosarcoma protuberans, other spindle cell tumors, and vascular lesions. (7506274)
1994
49
CD34 and factor XIIIa in the differential diagnosis of dermatofibroma and dermatofibrosarcoma protuberans. (7694515)
1993
50
Expression of the human progenitor cell antigen CD34 (HPCA-1) distinguishes dermatofibrosarcoma protuberans from fibrous histiocytoma in formalin-fixed, paraffin-embedded tissue. (7681857)
1993

Genetic Variations for Dermatofibrosarcoma

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Expression for genes affiliated with Dermatofibrosarcoma

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Dermatofibrosarcoma

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Pathways for genes affiliated with Dermatofibrosarcoma

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37NCBI BioSystems Database, 53Reactome, 12EMD Millipore, 51QIAGEN
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Compounds for genes affiliated with Dermatofibrosarcoma

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44Novoseek, 24HMDB, 49PharmGKB, 28IUPHAR, 11DrugBank, 2BitterDB, 59Tocris Bioscience
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Compounds related to Dermatofibrosarcoma according to GeneCards/GeneDecks:

(show top 50)    (show all 74)
idCompoundScoreTop Affiliating Genes
1estrogen4410.8F13A1
2procollagen4410.7F13A1, P4HTM
3glycogen44 2411.6ACTC1, KIT
4glucose4410.6ACTC1, MUC1, PDGFRB
5reticulin4410.5VIM, DES, MUC1, ACTC1
6creatinine4410.5ACTC1, APOD
7orcein4410.5VIM, S100B, DES, ACTC1
8eosin4410.5VIM, S100B, MUC1
92-methoxyethanol4410.4S100A1, ACTC1, VIM
10toluidine4410.4VIM, S100A1, KIT, DES
11fluorochrome4410.4VIM, KIT, CD34
12aspartate4410.3CTSK, COL1A1, EIF2AK3
13ganglioside4410.3DES, PDGFRB, VIM, NES
14serine4410.3DES, S100B, PDGFB, CTSK, TNFAIP6
15s 1004410.3VIM, ACTC1, MUC1, DES, F13A1, S100B
16acetylcholine44 49 28 11 2414.3NES, S100A1
17silver4410.3F13A1, PDGFB, S100A1, DES, CD34
18biotin44 11 2412.2S100A1, DES, MUC1, ACTC1, VIM
19chloramphenicol44 2 1112.2S100B, VIM, PDGFB, MUC1, COL1A1
20bromodeoxyuridine4410.2PDGFRB, DES, NES, PDGFB, S100B, VIM
21mucicarmine4410.2S100A1, VIM, S100B, DES, MUC1, ACTC1
22dexamethasone44 49 28 1113.2CD163, COL1A1, PDGFB, S100B, TNFAIP6
23formaldehyde44 2411.2MUC1, KIT, DES, S100B, VIM, ACTC1
24ribonucleic acid4410.2ACTC1, KIT, COL1A1, MUC1, PDGFRB, PDGFB
25agar4410.2PDGFB, VIM, MUC1, PDGFRB, CD34
26imatinib44 49 1112.2COL1A1, S100B, DES, PDGFRB, ACTC1, KIT
27matrigel4410.2NES, PDGFRB, VIM, ACTC1, MUC1
28b 7234410.1VIM, ACTC1, MUC1, S100B, S100A1, F13A1
29actomyosin4410.1S100A1, VIM, ACTC1
30methionine4410.0PDGFRB, S100B, VIM, DES, KIT
31thymidine44 2411.0S100B, COL1A1, CD34, KIT, MUC1, DES
3212-o-tetradecanoylphorbol 13-acetate4410.0MMP11, DES, CD34, MUC1, VIM
33iron44 2411.0F13A1, VIM, S100B, S100A1, ACTC1, DES
34proline4410.0DES, PDGFRB, MMP11, P4HTM, CTSK, S100B
35hyaluronic acid44 2411.0DES, TNFAIP6, VIM, MUC1
36testosterone44 59 11 2413.0MUC1, ACTC1, DES, KIT, S100B, PDGFRB
37oligonucleotide4410.0PDGFB, VIM, COL1A1, MMP11, KIT, F13A1
38dihydrotestosterone44 28 11 2412.9MUC1, DES, APOD, VIM
39oxygen44 2410.9P4HTM, EIF2AK3, MMP11, DES, NES, CTSK
40hematoxylin449.9DES, MUC1, CD34, KIT, ACTC1, F13A1
41nitric oxide44 11 2411.9S100B, NES, MUC1, S100A1, EIF2AK3, KIT
42agarose449.7APOD, MUC1, S100B, KIT
43lipid449.7PDGFB, MUC1, CD163, NES, APOD, PDGFRB
44cysteine449.7VIM, DES, MUC1, MMP11, COL1A1, S100B
45vegf449.5DES, PDGFRB, COL1A1, VIM, S100B, PDGFB
46tyrosine449.3S100A1, ACTC1, EIF2AK3, S100B, PDGFB, PDGFRB
47progesterone44 59 28 11 2413.3PDGFRB, APOD, NES, F13A1, DES, MUC1
48retinoic acid44 2410.2COL1A1, VIM, S100B, S100A1, PDGFRB, APOD
49calcium44 49 11 2412.2EIF2AK3, S100A1, COL1A1, KIT, ACTC1, CD163
50paraffin449.1S100A1, ACTC1, KIT, VIM, S100B, 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
1intermediate filamentGO:00588210.1DES, NES, VIM
2extracellular spaceGO:0056159.5COL1A1, S100B, PDGFB, APOD, CTSK, KIT
3extracellular regionGO:0055769.3MMP11, COL1A1, S100B, PDGFB, APOD, CTSK

Biological processes related to Dermatofibrosarcoma according to GeneCards/GeneDecks:

(show all 21)
idNameGO IDScoreTop Affiliating Genes
1metanephric glomerular mesangial cell proliferation involved in metanephros developmentGO:07226210.6PDGFB, PDGFRB
2positive regulation of metanephric mesenchymal cell migration by platelet-derived growth factor receptor-beta signaling pathwayGO:03579310.6PDGFRB, PDGFB
3paracrine signalingGO:03800110.6PDGFB, CD34
4intramembranous ossificationGO:00195710.5COL1A1, CTSK
5cell chemotaxisGO:06032610.5KIT, PDGFRB, PDGFB
6positive regulation of phosphatidylinositol 3-kinase activityGO:04355210.5KIT, PDGFRB, PDGFB
7positive regulation of DNA biosynthetic processGO:200057310.5PDGFRB, PDGFB
8positive regulation of phospholipase C activityGO:01086310.5PDGFRB, KIT
9positive regulation of phosphatidylinositol 3-kinase cascadeGO:01406810.5KIT, PDGFRB, PDGFB
10muscle filament slidingGO:03004910.5VIM, DES, ACTC1
11peptidyl-tyrosine phosphorylationGO:01810810.4KIT, PDGFRB, PDGFB
12stem cell proliferationGO:07208910.4NES, CD34
13positive regulation of chemotaxisGO:05092110.4PDGFRB, PDGFB
14positive regulation of calcium ion importGO:09028010.4PDGFRB, PDGFB
15hemopoiesisGO:03009710.4KIT, CD34, PDGFB
16collagen catabolic processGO:03057410.3COL1A1, CTSK, MMP11
17positive regulation of smooth muscle cell migrationGO:01491110.3PDGFRB, PDGFB
18extracellular matrix disassemblyGO:02261710.2COL1A1, CTSK, MMP11
19cardiac myofibril assemblyGO:05500310.2ACTC1, PDGFRB
20innate immune responseGO:0450879.9KIT, CTSK, PDGFRB, PDGFB, S100B
21extracellular matrix organizationGO:0301989.7COL1A1, PDGFB, CTSK, MMP11

Molecular functions related to Dermatofibrosarcoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1platelet-derived growth factor receptor bindingGO:00516110.3PDGFRB, PDGFB
2platelet-derived growth factor bindingGO:04840710.2PDGFRB, PDGFB, COL1A1
3S100 protein bindingGO:0445489.9S100A1, S100B
4identical protein bindingGO:0428029.7COL1A1, VIM, S100B, S100A1, DES, EIF2AK3

Products for genes affiliated with Dermatofibrosarcoma

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Sources for Dermatofibrosarcoma

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet