MCID: DRM004
MIFTS: 51

Dermatofibrosarcoma malady

Summaries for Dermatofibrosarcoma

Sources:
64Wikipedia, 33MalaCards
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Wikipedia:64 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 Osteoblast Signaling and Striated Muscle Contraction. The compounds estrogen and procollagen have been mentioned in the context of this disorder. Affiliated tissues include skin, lymph node and lung, and related mouse phenotypes are muscle and integument.

Aliases & Classifications for Dermatofibrosarcoma

Sources:
8Disease Ontology, 10DISEASES, 45Novoseek, 61UMLS, 35MeSH, 57SNOMED-CT
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Aliases & Descriptions:

dermatofibrosarcoma 8 10 45 61
dermatofibrosarcoma nos 8


External Ids:

Disease Ontology8 DOID:3507

Related Diseases for Dermatofibrosarcoma

Sources:
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 184)
idRelated DiseaseScoreTop Affiliating Genes
1dermatofibrosarcoma protuberans31.9S100A1, PDGFB, PDGFRB, F13A1, DES, MUC1
2fibrous histiocytoma30.4CD34
3cutaneous fibrous histiocytoma29.8MMP11, CD34
4choroid plexus papilloma29.8MUC1, S100B
5kaposi's sarcoma29.8PDGFB, F13A1, CD34
6wilms tumor29.8VIM, DES, ACTC1
7basal cell carcinoma29.8F13A1, CD34, MMP11, ACTC1
8spindle cell sarcoma29.8VIM, S100B, DES, MUC1, ACTC1
9gastrointestinal stromal tumor29.8VIM, S100B, S100A1, PDGFRB, NES, DES
10adenocarcinoma29.8VIM, PDGFB, PDGFRB, NES, F13A1, MUC1
11bednar's tumor10.3
12burn scar10.1
13merkel cell carcinoma10.1
14spindle cell lipoma10.1
15skin sarcoma10.1
16myxofibrosarcoma10.1
17hepatitis10.0PDGFB
18glomerulonephritis10.0PDGFB
19lymphangioleiomyomatosis10.0DES
20gliosarcoma10.0PDGFRB
21fasciitis10.0ACTC1
22leydig cell tumor10.0PDGFRB
23uterine carcinosarcoma10.0KIT
24infantile myofibromatosis10.0DES
25renal oncocytoma10.0S100A1
26dermatitis10.0F13A1
27malignant fibroxanthoma10.0CD34
28neurilemmoma10.0CD34
29stomach cancer10.0CD34
30carney triad10.0KIT
31papilloma10.0ACTC1
32hemangioendothelioma10.0CD34
33cholangiocarcinoma10.0MUC1
34liver cirrhosis10.0PDGFB
35adenomatoid tumor10.0VIM
36sertoli-leydig cell tumor10.0MUC1
37histiocytosis10.0CD163, S100B
38endometrial carcinoma10.0CD34, APOD
39pneumonia10.0MUC1
40lung cancer10.0CD34, MUC1
41juvenile xanthogranuloma10.0S100B, F13A1
42fibrous meningioma10.0S100B, MUC1
43pituitary adenoma10.0NES
44pulmonary embolism10.0F13A1
45oligodendroglioma10.0PDGFRB, APOD
46cystadenocarcinoma10.0MUC1
47adenosarcoma10.0DES, ACTC1
48thymic carcinoma10.0MUC1, KIT
49endometriosis10.0ACTC1, MMP11
50mastocytosis10.0KIT, PDGFRB

Graphical network of the top 20 diseases related to Dermatofibrosarcoma:



Diseases related to dermatofibrosarcoma

Clinical Features for Dermatofibrosarcoma

Drugs & Therapeutics for Dermatofibrosarcoma

Sources:
5CenterWatch, 42NIH Clinical Center, 6ClinicalTrials, 61UMLS, 41NDF-RT
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Approved drugs:

Search CenterWatch for Dermatofibrosarcoma

Drug clinical trials:

Search ClinicalTrials for Dermatofibrosarcoma

Search NIH Clinical Center for Dermatofibrosarcoma

Search CenterWatch for Dermatofibrosarcoma

Genetic Tests for Dermatofibrosarcoma

Anatomical Context for Dermatofibrosarcoma

Sources:
33MalaCards
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MalaCards organs/tissues related to Dermatofibrosarcoma:

33
Skin, Lymph node, Lung, Thyroid, Breast, T cells, B cells, Endothelial, Fetal lung, Fetal thyroid

Animal Models for Dermatofibrosarcoma or affiliated genes

Sources:
37MGI, 28inGenious Targeting Laboratory
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Publications for Dermatofibrosarcoma

Sources:
51PubMed
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Articles related to Dermatofibrosarcoma:

(show top 50)    (show all 537)
idTitleAuthorsYear
1
Dermatofibrosarcoma protuberans: Margins reduction using slow-Mohs micrographic surgery. Experience with 35 patients. (24411817)
2014
2
Artificial dermis (MatridermAr) followed by skin graft as an option in dermatofibrosarcoma protuberans with complete circumferential and peripheral deep margin assessment. (24102765)
2013
3
Atrophic dermatofibrosarcoma protuberans: a case report. (23113648)
2013
4
Molecular and clinicopathological analysis of dermatofibrosarcoma protuberans. (23207290)
2013
5
Dermatofibrosarcoma protuberans: Our experience of 59 cases. (23162649)
2012
6
Half forehead reconstruction with a single rotational scalp flap for dermatofibrosarcoma protuberans treatment. (22559860)
2012
7
Dermatofibrosarcoma protuberans in a child: a case report. (23316399)
2012
8
Multidisciplinary approach to the management of dermatofibrosarcoma protuberans. (22387033)
2012
9
Dermatofibrosarcoma protuberans arising at a Rho(D) immune globulin injection site. (23270191)
2012
10
Dermatofibrosarcoma protuberans: an unusual case of neck swelling. (23866454)
2012
11
Using the "feed and wrap" technique and Mohs surgery to eradicate congenital dermatofibrosarcoma protuberans in a 4-month-old. (21605252)
2011
12
A case of congenital pigmented dermatofibrosarcoma protuberans (Bednar tumor) in a patient with Fanconi anemia. (21438918)
2011
13
Dermatofibrosarcoma protuberans of the scalp with fibrosarcomatous degeneration and pulmonary metastasis. (22267990)
2011
14
Imaging of dermatofibrosarcoma protuberans of breast. (20626395)
2010
15
Wide excision or Mohs micrographic surgery for the treatment of primary dermatofibrosarcoma protuberans. (19858696)
2010
16
Dermatofibrosarcoma protuberans recurring as a hybrid dermatofibrosarcoma/giant cell fibroblastoma in an adult: a case report. (20655288)
2010
17
GIST with a twist--upregulation of PDGF-B resulting in metachronous gastrointestinal stromal tumor and dermatofibrosarcoma protuberans. (19936851)
2010
18
Dermatofibrosarcoma protuberans: how wide should we resect? (20354798)
2010
19
Using imatinib as neoadjuvant therapy in dermatofibrosarcoma protuberans: potential pluses and minuses. (20870634)
2010
20
Recurrence of dermatofibrosarcoma protuberans in post-surgical scar detected by 18F-FDG-PET imaging. (19330190)
2009
21
Sonography of dermatofibrosarcoma protuberans of the groin. (18431749)
2008
22
Dermatofibrosarcoma protuberans of the face: report of 2 cases and an overview of the recent literature. (16360873)
2006
23
Analysis of the COL1A1-PDGFB fusion gene in a case of dermatofibrosarcoma protuberans with a fibrosarcoma component]. (16956568)
2006
24
The expression levels and the differential expression of transforming growth factor-beta receptors in dermatofibroma and dermatofibrosarcoma protuberans. (16634896)
2006
25
Dermatofibrosarcoma protuberans occuring in a child. (15943042)
2005
26
Imatinib mesylate: an attractive alternative in young children with large, surgically challenging dermatofibrosarcoma protuberans. (15503291)
2005
27
Dermatofibrosarcoma protuberans of the forehead and scalp with involvement of the outer calvarial plate: multistaged repair with the use of skin expanders. (15720109)
2005
28
Dermatofibrosarcoma protuberans treated at a single institution: a surgical disease with a high cure rate. (16234529)
2005
29
Dermatofibrosarcoma protuberans of the face: surgical management. (15915111)
2005
30
Apo D in soft tissue tumors: a novel marker for dermatofibrosarcoma protuberans. (15252314)
2004
31
Atrophic dermatofibrosarcoma protuberans: an uncommon and misleading variant. (15250897)
2004
32
Outcomes of surgery for dermatofibrosarcoma protuberans. (15028319)
2004
33
Dermatofibrosarcoma protuberans, giant cell fibroblastoma, and hybrid lesions in children: clinicopathologic comparative analysis of 28 cases with molecular data--a study from the French Federation of Cancer Centers Sarcoma Group. (12502925)
2003
34
Dermatofibrosarcoma protuberans. (12656008)
2003
35
Reconstruction of a scalp and skull defect with free latissimus dorsi myocutaneous flap following dermatofibrosarcoma protuberans. (12493936)
2002
36
Pediatric pigmented dermatofibrosarcoma protuberans (BednA!r tumor): case report and review of the literature with emphasis on the differential diagnosis. (12440785)
2002
37
Concomitant DNA copy number amplification at 17q and 22q in dermatofibrosarcoma protuberans. (11435686)
2001
38
Dermatofibrosarcoma protuberans of scalp : a case report. (11303249)
2001
39
Structural and functional analysis of a chimeric protein COL1A1-PDGFB generated by the translocation t(17;22)(q22;q13.1) in Dermatofibrosarcoma protuberans (DP). (11420709)
2001
40
A morphologic study of dermatofibrosarcoma protuberans: expansion of a histologic profile. (10774935)
2000
41
Indeterminate fibrohistiocytic lesions of the skin: is there a spectrum between dermatofibroma and dermatofibrosarcoma protuberans? (10895822)
2000
42
Dermatofibrosarcoma protuberans of the vulva: a case report and review of the literature. (10873415)
2000
43
Dermatofibrosarcoma protuberans: early recognition and treatment. (11011855)
2000
44
Dermatofibrosarcoma protuberans: what is the best surgical approach? (11128831)
1999
45
Dermatofibrosarcoma protuberans of the vulva: clinicopathologic and immunohistochemical analysis of four cases, one with fibrosarcomatous change, and review of the literature. (10542946)
1999
46
Sclerosing dermatofibrosarcoma protuberans. (9826170)
1998
47
Sarcomatous dermatofibrosarcoma protuberans metastasized to the lung: preservation of CD34 expression in tumor cells. (9952345)
1998
48
Composite tumor consisting of dermatofibrosarcoma protuberans and myxofibrosarcoma. (9826171)
1998
49
Dermatofibrosarcoma protuberans treated with Mohs surgery. A case with CD34 immunostaining variability. (8608381)
1996
50
Sarcomas--a clinicopathological guide with particular reference to cutaneous manifestation. I. Dermatofibrosarcoma protuberans, malignant fibrous histiocytoma and the epithelioid sarcoma of Enzinger. (6091955)
1984

Genetic Variations for Dermatofibrosarcoma

Expression for genes affiliated with Dermatofibrosarcoma

Sources:
1BioGPS, 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

Sources:
38NCBI BioSystems Database, 54Reactome, 12EMD Millipore, 52QIAGEN
See all sources

Compounds for genes affiliated with Dermatofibrosarcoma

Sources:
45Novoseek, 24HMDB, 50PharmGKB, 29IUPHAR, 11DrugBank, 2BitterDB, 60Tocris Bioscience
See all sources

Compounds related to Dermatofibrosarcoma according to GeneCards/GeneDecks:

(show top 50)    (show all 74)
idCompoundScoreTop Affiliating Genes
1estrogen4510.8F13A1
2procollagen4510.7P4HTM, F13A1
3glycogen45 2411.6KIT, ACTC1
4glucose4510.6PDGFRB, MUC1, ACTC1
5reticulin4510.5VIM, DES, MUC1, ACTC1
6creatinine4510.5APOD, ACTC1
7orcein4510.5VIM, S100B, DES, ACTC1
8eosin4510.5VIM, S100B, MUC1
92-methoxyethanol4510.4ACTC1, S100A1, VIM
10toluidine4510.4KIT, DES, S100A1, VIM
11fluorochrome4510.4VIM, CD34, KIT
12aspartate4510.3EIF2AK3, CTSK, COL1A1
13ganglioside4510.3VIM, PDGFRB, NES, DES
14s 1004510.3VIM, S100B, F13A1, DES, MUC1, ACTC1
15serine4510.3S100B, PDGFB, TNFAIP6, CTSK, DES
16acetylcholine45 50 29 11 2414.3NES, S100A1
17silver4510.3S100A1, PDGFB, F13A1, DES, CD34
18chloramphenicol45 2 1112.2COL1A1, VIM, S100B, PDGFB, MUC1
19biotin45 11 2412.2VIM, S100A1, DES, MUC1, ACTC1
20bromodeoxyuridine4510.2VIM, S100B, PDGFB, PDGFRB, NES, DES
21mucicarmine4510.2VIM, S100B, S100A1, DES, MUC1, ACTC1
22dexamethasone45 50 29 1113.2COL1A1, S100B, PDGFB, TNFAIP6, CD163
23formaldehyde45 2411.2VIM, S100B, DES, MUC1, KIT, ACTC1
24ribonucleic acid4510.2COL1A1, PDGFB, PDGFRB, MUC1, KIT, ACTC1
25agar4510.2VIM, PDGFB, PDGFRB, MUC1, CD34
26imatinib45 50 1112.2COL1A1, S100B, PDGFB, PDGFRB, DES, KIT
27matrigel4510.2ACTC1, MUC1, NES, PDGFRB, VIM
28b 7234510.1ACTC1, MUC1, DES, F13A1, S100A1, S100B
29actomyosin4510.1ACTC1, S100A1, VIM
30methionine4510.0VIM, S100B, PDGFRB, DES, KIT
31thymidine45 2411.0COL1A1, S100B, PDGFB, DES, MUC1, CD34
3212-o-tetradecanoylphorbol 13-acetate4510.0VIM, DES, MUC1, CD34, MMP11
33iron45 2411.0VIM, S100B, S100A1, F13A1, DES, CD163
34proline4510.0VIM, S100B, PDGFRB, CTSK, DES, P4HTM
35hyaluronic acid45 2411.0MUC1, DES, TNFAIP6, VIM
36testosterone45 60 11 2413.0VIM, S100B, PDGFRB, APOD, DES, MUC1
37oligonucleotide4510.0COL1A1, VIM, PDGFB, PDGFRB, APOD, F13A1
38dihydrotestosterone45 29 11 2412.9VIM, APOD, DES, MUC1
39oxygen45 2410.9S100B, CTSK, NES, DES, P4HTM, MUC1
40hematoxylin459.9KIT, ACTC1, CD34, MUC1, DES, VIM
41nitric oxide45 11 2411.9S100B, S100A1, PDGFB, NES, DES, MUC1
42agarose459.7S100B, APOD, MUC1, KIT
43lipid459.7VIM, S100B, PDGFB, PDGFRB, APOD, NES
44cysteine459.7COL1A1, VIM, S100B, S100A1, PDGFRB, CTSK
45vegf459.5VIM, COL1A1, S100B, PDGFB, ACTC1, KIT
46tyrosine459.3TNFAIP6, PDGFRB, PDGFB, S100A1, S100B, CTSK
47progesterone45 60 29 11 2413.3VIM, S100B, S100A1, PDGFB, PDGFRB, APOD
48retinoic acid45 2410.2ACTC1, COL1A1, VIM, S100B, S100A1, PDGFRB
49calcium45 50 11 2412.2CTSK, TNFAIP6, PDGFRB, S100A1, S100B, COL1A1
50paraffin459.1S100A1, PDGFB, PDGFRB, APOD, NES, F13A1

GO Terms for genes affiliated with Dermatofibrosarcoma

Sources:
16Gene Ontology
See all sources

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

3CDC
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
51PubMed
52QIAGEN
58SNOMED-CT via Orphanet
61UMLS
62UMLS via Orphanet