Dermatofibrosarcoma Protuberans malady

NIH Rare Diseases: Dermatofibrosarcoma protuberans is an uncommon tumor that arises in the skin. The tumor is firm and often flesh-colored although it can be reddish, bluish, or purplish. The tumor is often found on the chest or shoulders, but it can be found on other parts of the body.  Dermatofibrosarcoma protuberans may cause no symptoms, and the initial size of the tumor tends to be around 1 to 5 centimeters. This tumor has a low potential to spread to other tissues (metastasize). Treatment often involves surgery to remove the tumor, such as by Mohs’ micrographic surgery. ³⁰

MalaCards: Dermatofibrosarcoma Protuberans, also known as DFSP, is related to bednar's tumor and fibrous histiocytoma. An important gene associated with Dermatofibrosarcoma Protuberans is PDGFB (platelet-derived growth factor beta polypeptide), and among its related pathways are Breast Cancer Regulation by Stathmin1 and Cytoskeleton remodeling_Role of PDGFs in cell migration. The drugs prednisone and prednisolone tebutate and the compounds egcg and orcein have been mentioned in the context of this disorder. Affiliated tissues include lung, breast and skin, and related mouse phenotypes are respiratory system and endocrine/exocrine gland.

Genetics Home Reference: Dermatofibrosarcoma protuberans is a rare type of cancer that causes a tumor in the deep layers of skin. This condition is a type of soft tissue sarcoma, which are cancers that affect skin, fat, muscle, and similar tissues.¹⁷

Wikipedia: Dermatofibrosarcoma protuberans (DFSP) is a very rare tumor. It is a rare neoplasm of the dermis layer...⁴⁴ more...

dermatofibrosarcoma protuberans 7 30 17 33 32 43 dfsp 30 17 metastatic dermatofibrosarcoma protuberans (subtype) 30 familial dermatofibrosarcoma protuberans (subtype) 30 giant-cell fibroblastoma 33

giant cell fibroblastoma 30 darier-hoffmann tumor 17 darier-ferrand tumor 17 neoplasms 43

Diseases related to dermatofibrosarcoma protuberans by text searches and GeneDecks gene sharing:

(show top 50)    (show all 1210)

id	Related Disease	Score	Top Affiliating Genes
1	bednar's tumor	33.6	VIM, S100B, ENO2, COL1A1, PDGFB
2	fibrous histiocytoma	32.8	VIM, S100B, CD163, CD34, F13A1, ACTC1
3	ring chromosomes	32.3	HMGA1, HMGA2, COL1A1, PDGFB
4	intraepithelial neoplasm	30.3	MUC1, TP53, EIF2AK3
5	tongue cancer	30.2	VIM, MMP1, MMP2, TP53
6	nasopharynx carcinoma	29.7	MMP3, ETS1, TP53, TGFBR2
7	bone cancer	29.4	ENO2, CTSK, COL1A1, DES
8	pancreatic cancer	29.3	MUC1, MMP14, PDGFRB, TGFBR2, NES
9	sex cord-gonadal stromal tumor	29.1	VIM, MUC1, S100B, DES
10	brain cancer	29.0	MMP14, ENO2, TP53, PDGFB, PDGFRA, PDGFRB
11	myoma	28.8	HMGA2, PTGS2, DES
12	liver cancer	28.7	MMP14, TP53, PTGS2, PDGFB, PDGFRA, TGFBR2
13	choroid plexus carcinoma	28.2	VIM, TP53
14	lung benign neoplasm	28.2	VIM, ENO2
15	lipoma	28.1	VIM, S100A1, CD34, MMP3, HMGA1, HMGA2
16	adenoid cystic carcinoma	27.8	KIT, VIM, MUC1, S100A1, S100B, PDGFRB
17	perivascular epithelioid cell tumor	27.8	KIT, VIM, MUC1, S100A1, S100B, ACTC1
18	myelodysplastic syndrome	27.7	KIT, CD34, PDGFRA, PDGFRB, ABL1
19	breast cancer	27.7	MMP1, MMP2, MMP3, TP53, PTGS2
20	angiomyolipoma	27.4	KIT, VIM, MUC1, S100A1, S100B, TP53
21	glomus tumor	27.3	VIM, MUC1, S100A1, S100B, CD34, ACTC1
22	basal cell carcinoma	27.2	CD34, MMP1, MMP11, MMP3, F13A1, TP53
23	giant cell tumor	27.1	VIM, MUC1, S100B, F13A1, CTSK, ACTC1
24	mucinous cystadenocarcinoma	27.1	VIM, MUC1, ACTC1
25	mixed cell type cancer	27.0	VIM, ENO2, TP53, DES
26	hemangioendothelioma	26.9	S100B, CD34, F13A1
27	kidney cancer	26.9	KIT, VIM, MUC1, TP53, PDGFRB
28	chromophobe renal cell carcinoma	26.9	KIT, VIM, MUC1, S100A1
29	renal cell carcinoma	26.9	KIT, VIM, MUC1, S100A1, MMP2, ENO2
30	esophageal cancer	26.8	MUC1, MMP11, MMP2, ETS1, TP53, PTGS2
31	pleomorphic adenoma	26.7	VIM, MUC1, S100A1, S100B, HMGA1, HMGA2
32	anaplastic carcinoma	26.7	VIM, MUC1, ENO2
33	perineurioma	26.7	KIT, VIM, MUC1, S100B, CD34, F13A1
34	malignant peripheral nerve sheath tumor	26.7	KIT, VIM, MUC1, S100A1, S100B, CD34
35	craniopharyngioma	26.7	VIM, S100A1, CD34, TP53, PDGFB, PDGFRA
36	polymorphous low-grade adenocarcinoma	26.6	KIT, VIM, MUC1, S100A1, S100B, ACTC1
37	spiradenoma	26.6	S100A1, TP53, ACTC1
38	kaposi's sarcoma	26.6	KIT, VIM, P4HTM, CD34, MMP2, F13A1
39	granular cell tumor	26.5	VIM, S100A1, S100B, ENO2, F13A1, DES
40	mastocytosis	26.4	KIT, VIM, PDGFRA, PDGFRB, ACTC1
41	dermatofibrosarcoma	26.3	KIT, VIM, MUC1, S100A1, S100B, P4HTM
42	neuroendocrine tumor	26.3	KIT, MUC1, S100B, ENO2, TP53, PTGS2
43	paraganglioma	26.3	KIT, VIM, S100A1, S100B, MMP2, ENO2
44	adenomatoid tumor	26.3	VIM, MUC1, MMP2, DES
45	synovial sarcoma	26.1	KIT, VIM, MUC1, S100B, HMGA2, DES
46	cystadenoma	26.0	KIT, VIM, MUC1, S100A1, TP53, ACTC1
47	seminoma	25.9	KIT, VIM, ENO2, TP53, PDGFRA, DES
48	adenoid squamous cell carcinoma	25.9	VIM, MUC1, CD34
49	malignant giant cell tumor of soft parts	25.9	S100B, ACTC1, DES
50	glomangioma	25.9	S100A1, ACTC1, DES

Approved drugs:

Drug clinical trials:

Inferred drug relations via UMLS/NDF-RT:

^43 28 dexamethasone, dexamethasone acetate, dexamethasone acetate pwdr [va product], dexamethasone phosphate, dexamethasone sodium phosphate, dexamethasone sodium phosphate pwdr [va product], linolenic acid, methylprednisolone, methylprednisolone acetate, methylprednisolone sodium succinate, prednisolone, prednisolone acetate, prednisolone acetate pwdr [va product], prednisolone pwdr [va product], prednisolone sodium phosphate, prednisolone tebutate, prednisone, prednisone pwdr [va product]

MalaCards organs/tissues related to dermatofibrosarcoma protuberans:

²²

MGI Mouse Phenotypes related to dermatofibrosarcoma protuberans:

²⁵ (show all 25)

id	Description	MGI Source Accession	Score	Top Affiliating Genes
1	respiratory system phenotype	MP:0005388	10.6	PDGFB, F13A1
2	endocrine/exocrine gland phenotype	MP:0005379	9.8	PSIP1, CTSK, HMGA2, HTT
3	craniofacial phenotype	MP:0005382	9.1	PSIP1, PDGFRB, PDGFRA, CTSK, HMGA2, HTT
4	embryogenesis phenotype	MP:0005380	8.8	ABL1, PDGFRA, COL1A1, ETS1, HTT, VIM
5	pigmentation phenotype	MP:0001186	8.7	SOX2, PDGFRA, PDGFB, COL1A1, TP53, KIT
6	integument phenotype	MP:0010771	8.5	PDGFRA, COL1A1, TP53, HMGA2, HTT, CD34
7	normal phenotype	MP:0002873	8.4	SOX2, PSIP1, TGFBR2, ABL1, PDGFRA, COL1A1
8	hematopoietic system phenotype	MP:0005397	8.3	PSIP1, ABL1, PDGFRA, PDGFB, COL1A1, CTSK
9	limbs/digits/tail phenotype	MP:0005371	8.3	EIF2AK3, PDGFRA, COL1A1, CTSK, TP53, HMGA2
10	no phenotypic analysis	MP:0003012	8.0	SOX2, PDGFB, COL1A1, PTGS2, TP53, HMGA1
11	adipose tissue phenotype	MP:0005375	8.0	TGFBR2, PDGFRB, COL1A1, PTGS2, TP53, HMGA2
12	reproductive system phenotype	MP:0005389	7.8	SOX2, PSIP1, PDGFRA, PDGFB, PTGS2, TNFAIP6
13	nervous system phenotype	MP:0003631	7.6	NES, SOX2, EIF2AK3, ABL1, PDGFRA, COL1A1
14	immune system phenotype	MP:0005387	7.0	PDGFRA, PDGFRB, ABL1, EIF2AK3, PSIP1, SOX2
15	tumorigenesis	MP:0002006	7.0	TGFBR2, PDGFRA, COL1A1, PTGS2, TP53, HMGA1
16	behavior/neurological phenotype	MP:0005386	6.8	PDGFRA, ABL1, EIF2AK3, TGFBR2, DES, PSIP1
17	digestive/alimentary phenotype	MP:0005381	6.7	PDGFRB, ABL1, EIF2AK3, TGFBR2, SOX2, PDGFRA
18	mortality/aging	MP:0010768	6.6	EIF2AK3, TGFBR2, DES, PSIP1, SOX2, NES
19	growth/size phenotype	MP:0005378	6.5	PDGFRA, PDGFRB, ABL1, EIF2AK3, ACTC1, TGFBR2
20	vision/eye phenotype	MP:0005391	6.4	SOX2, PSIP1, TGFBR2, ABL1, PDGFRB, PDGFB
21	skeleton phenotype	MP:0005390	6.2	PSIP1, TGFBR2, EIF2AK3, ABL1, CTSK, PTGS2
22	muscle phenotype	MP:0005369	6.1	PDGFRA, PDGFRB, ABL1, ACTC1, TGFBR2, DES
23	cellular phenotype	MP:0005384	5.3	PDGFRB, ABL1, EIF2AK3, ACTC1, TGFBR2, PSIP1
24	cardiovascular system phenotype	MP:0005385	5.2	PTGS2, COL1A1, PDGFB, PDGFRA, PDGFRB, ABL1
25	homeostasis/metabolism phenotype	MP:0005376	3.6	KIT, PTGS2, CTSK, COL1A1, PDGFB, PDGFRA

Articles related to dermatofibrosarcoma protuberans:

(show top 50)    (show all 66)

id	Title	Authors	Year	Affiliating Genes
1	Cathepsin K expression: a useful marker for the diffe rential diagnosis of dermatofibroma and dermatofibrosarcoma protuberans. (20727023)	Yan X.... Furue M.	2010	CTSK
2	Plaque-like CD34-positive dermal fibroma ('medal lion-like dermal dendrocyte hamartoma'): clinicopathologic, immunohistoche mical, and molecular analysis of 5 cases emphasizing its distinction from super ficial, plaque-like dermatofibrosarcoma protuberans. (20061935)	Kutzner H.... Requena L.	2010	COL1A1, PDGFB
3	Dermatofibrosarcoma protuberans of the vulva: a clini copathologic and immunohistochemical study of 13 cases. (20139758)	Edelweiss M.... Malpica A.	2010	PDGFRA, PDGFRB, ABL1
4	PDGFB quantification is a useful tool in the diagnosi s of dermatofibrosarcoma protuberans: a study of 10 cases. (19663837)	Muchemwa F.C.... Ihn H.	2010	COL1A1, PDGFB
5	Dermatofibrosarcoma protuberans: clinical, pathologic al, and genetic (COL1A1-PDGFB ) study with therapeutic implications. (19635106)	Llombart B.... GuillAcn C.	2009	COL1A1, PDGFB
6	Cyclooxygenase-2 expression in dermatofibroma and dermatofibrosarcoma protuberans. (18201240)	Adler N.... Feinmesser M.	2008	PTGS2
7	Expression of nestin in dermatofibrosarcoma protuberans in comparison to dermatofibroma. (18705829)	Mori T.... Narisawa Y.	2008	F13A1, NES
8	Apolipoprotein D in CD34-positive and CD34-negative cutaneous neoplasms: a useful marker in differentiating superficial acral fibromyxoma from dermatofibrosarcoma protuberans. (17885669)	Lisovsky M.... Mahalingam M.	2008	CD34, APOD
9	Dermatofibrosarcoma protuberans COL1A1-PDGFB fusion is identified in virtually all dermatofibrosarcoma protuberans cases when investigated by newly developed multiplex reverse transcription polymerase chain reaction and fluorescence in situ hybridization assays. (17950782)	Patel K.U.... Lopez-Terrada D.	2008	COL1A1, PDGFB
10	Genomic gains of COL1A1-PDFGB occur in the histologic evolution of giant cell fibroblastoma into dermatofibrosarcoma protuberans. (18069662)	Macarenco R.S.... Oliveira A.M.	2008	COL1A1, PDGFB
11	A novel fusion gene of collagen type I alpha 1 (exon 31) and platelet-derived growth factor B-chain (exon 2) in dermatofibrosarcoma protuberans. (17478383)	Nakanishi G.... Iwatsuki K.	2007	COL1A1
12	Dermatofibrosarcoma protuberans: clinicopathological aspects of an unusual cutaneous tumor. (17595787)	Korkolis D.P.... Vassilopoulos P.P.	2007	CD34
13	From cytogenetics to cytogenomics of dermatofibrosarcoma protuberans family of tumors (17337387)	Bianchini L.... Pedeutour F.	2007	COL1A1
14	The expression levels and the differential expression of transforming growth factor-beta receptors in dermatofibroma and dermatofibrosarcoma protuberans. (16634896)	Kubo M.... Tamaki K.	2006	TGFBR2
15	Dermatofibrosarcoma protuberans: a surgical disease with a molecular savior. (16721128)	McArthur G.A.	2006	COL1A1, PDGFB
16	Overexpression of phosphorylated-STAT3 and phosphorylated-ERK protein in dermatofibrosarcoma protuberans. (16709490)	Lin N.... Furue M.	2006	EIF2AK3
17	Analysis of gene mutations in four cases of dermatofibrosarcoma protuberans. (16681596)	Saeki H.... Tamaki K.	2006	COL1A1, PDGFB
18	Expression of CD163 in dermatofibroma, cellular fibrous histiocytoma, and dermatofibrosarcoma protuberans: comparison with CD68, CD34, and Factor XIIIa. (16640542)	Sachdev R.... Sundram U.	2006	F13A1, CD163
19	Gene mutation analysis in five cases of dermatofibrosarcoma protuberans using formalin-fixed, paraffin-embedded tissues. (16040406)	Saeki H.... Tamaki K.	2005	COL1A1, PDGFB
20	Molecular and clinical analysis of locally advanced dermatofibrosarcoma protuberans treated with imatinib: Imatinib Target Exploration Consortium Study B2225. (15681532)	McArthur G.A.... Fletcher J.A.	2005	PDGFB
21	Differential expression of HMGA1 and HMGA2 in dermatofibroma and dermatofibrosarcoma protuberans: potential diagnostic applications, and comparison with histologic findings, CD34, and factor XIIIa immunoreactivity. (15249855)	Li N.... Tallini G.	2004	F13A1, HMGA1, HMGA2
22	Dermatofibrosarcoma protuberans--an update. (16279228)	Breuninger H.... Garbe C.	2004	F13A1
23	Apo D in soft tissue tumors: a novel marker for dermatofibrosarcoma protuberans. (15252314)	West R.B.... Lui C.L.	2004	APOD
24	Microsatellite instability and p53 mutation associated with tumor progression in dermatofibrosarcoma protuberans. (14991543)	Takahira T.... Tsuneyoshi M.	2004	TP53
25	Immunohistochemical expression of matrix metalloproteinases 1, 2, 9, and 14 in dermatofibrosarcoma protuberans and common fibrous histiocytoma (dermatofibroma). (15387709)	Weinrach D.M.... Laskin W.B.	2004	MMP3, MMP2, MMP1
26	Dermatofibrosarcoma protuberans and breast cancer: genetic link or coincidental association? (15212412)	Beech D.J.... Long W.P.	2004	TP53
27	CD44 and hyaluronate in the differential diagnosis of dermatofibroma and dermatofibrosarcoma protuberans. (12641778)	Calikoglu E.... Kaya G.	2003	HABP2, TNFAIP6
28	Dermatofibrosarcoma protuberans of breast. (12660034)	Sandberg A.A.... Hashimoto H.	2003	COL1A1, PDGFB
29	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)	Saeki H.... Tamaki K.	2003	COL1A1, PDGFB
30	Genetics of dermatofibrosarcoma protuberans family of tumors: from ring chromosomes to tyrosine kinase inhibitor treatment. (12661001)	Sirvent N.... Pedeutour F.	2003	COL1A1
31	EMA+ cells in dermatofibrosarcoma protuberans. A stud y of 11 tumors suggesting perineurial cell differentiation. (12426982)	ZA!mecnA-k M.... Michal M.	2002	MUC1
32	COLIA1-PDGFB gene fusion in dermatofibrosarcoma protuberans. molecular analysis of a case with an unusual large marker containing sequences from chromosomes 7, 8, 17, 21, and 22. (12127408)	Maire G.... Limon J.	2002	COL1A1, PDGFB
33	Fusion of COL1A1 exon 29 with PDGFB exon 2 in a der(22)t(17;22) in a pediatric giant cell fibroblastoma with a pigmented Bednar tumor component. Evidence for age-related chromosomal pattern in dermatofibrosarcoma protuberans and related tumors. (12034531)	Maire G.... Pedeutour F.	2002	COL1A1, PDGFB
34	Tenascin differentiates dermatofibroma from dermatofibrosarcoma protuberans: comparison with CD34 and factor XIIIa. (11172295)	Kahn H.J.... From L.	2001	F13A1
35	Growth inhibition of dermatofibrosarcoma protuberans tumors by the platelet-derived growth factor receptor antagonist STI571 through induction of apoptosis. (11479215)	SjAPblom T.... Heldin C.H.	2001	PDGFB
36	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)	Simon M.P.... Pouyssegur J.	2001	PDGFB
37	Indeterminate fibrohistiocytic lesions of the skin: is there a spectrum between dermatofibroma and dermatofibrosarcoma protuberans? (10895822)	Horenstein M.G.... Shea C.R.	2000	F13A1
38	Adjuvant therapy with hyaluronidase prior to excision of dermatofibrosarcoma protuberans. (10193968)	Menon P.... Skelton H.	1999	HTT
39	The dermatofibrosarcoma protuberans-associated collagen type Ialpha1/platelet-derived growth factor (PDGF) B-chain fusion gene generates a transforming protein that is processed to functional PDGF-BB. (10446987)	Shimizu A.... Ostman A.	1999	COL1A1, PDGFB
40	Accelerated growth of dermatofibrosarcoma protuberans during pregnancy. (10534646)	Parlette L.E.... Skelton H.	1999	F13A1
41	An ultrastructural and immunohistochemical study of p igmented dermatofibrosarcoma protuberans (Bednar tumor). (10417439)	Kagoura M.... Morohashi M.	1999	S100B, VIM, ENO2
42	Pretreatment with intralesional hyaluronidase prior to excision of dermatofibrosarcoma protuberans. (9839627)	Smith K.... Skelton H.	1998	HTT
43	Transforming activity of the chimeric sequence formed by the fusion of collagen gene COL1A1 and the platelet derived growth factor b-chain gene in dermatofibrosarcoma protuberans. (9771975)	Greco A.... Pierotti M.A.	1998	COL1A1
44	Pigmented dermatofibrosarcoma protuberans (BednA!r tumor) occurring in a Japanese infant. (9693186)	Kaburagi Y.... Takehara K.	1998	F13A1
45	Deregulation of the platelet-derived growth factor B-chain gene via fusion with collagen gene COL1A1 in dermatofibrosarcoma protuberans and giant-cell fibroblastoma. (8988177)	Simon M.-P.... Dumanski J.P.	1997	COL1A1, PDGFB
46	CD34 and factor-XIIIa immunoreactivity in dermatofibrosarcoma protuberans and dermatofibroma. (9129699)	Goldblum J.R.... Tuthill R.J.	1997	CD34, F13A1
47	Dermatofibrosarcoma protuberans treated with Mohs surgery. A case with CD34 immunostaining variability. (8608381)	Garcia C.... Clark R.E.	1996	CD34
48	Atrophic variants of dermatofibroma and dermatofibrosarcoma protuberans. (7545142)	Zelger B.W.... Zelger B.G.	1995	F13A1
49	Expression of the human hematopoietic progenitor cell antigen CD34 in dermatofibrosarcoma protuberans, other spindle cell tumors, and vascular lesions. (7506274)	Cohen P.R.... Farhood A.I.	1994	CD34
50	Dermatofibroma and dermatofibrosarcoma protuberans: differential expression of CD34 and factor XIIIa. (7528477)	Cohen P.R.... Farhood A.I.	1994	F13A1

Genes related to dermatofibrosarcoma protuberans according to GeneCards:

(show all 37)

id	Symbol	Description	Score	GeneCards Section Context
1	PDGFB	platelet-derived growth factor beta polypeptide	11.1	Summaries, Publications
2	COL1A1	collagen, type I, alpha 1	11.1	Publications, Summaries
3	MMP11	matrix metallopeptidase 11 (stromelysin 3)	11.0	Publications
4	CD34	CD34 molecule	11.0	Publications
5	F13A1	coagulation factor XIII, A1 polypeptide	11.0	Publications
6	S100A1	S100 calcium binding protein A1	11.0
7	PDGFRB	platelet-derived growth factor receptor, beta polypeptide	11.0	Publications
8	ACTC1	actin, alpha, cardiac muscle 1	11.0
9	VIM	vimentin	11.0	Publications
10	S100B	S100 calcium binding protein B	11.0	Publications
11	DES	desmin	11.0
12	MUC1	mucin 1, cell surface associated	11.0	Publications
13	KIT	v-kit Hardy-Zuckerman 4 feline sarcoma viral oncogene homolog	11.0
14	P4HTM	prolyl 4-hydroxylase, transmembrane (endoplasmic reticulum)	11.0	Publications
15	TNFAIP6	tumor necrosis factor, alpha-induced protein 6	11.0	Publications
16	APOD	apolipoprotein D	11.0	Publications
17	CD163	CD163 molecule	11.0	Publications
18	SOX2	SRY (sex determining region Y)-box 2	11.0	Publications
19	HABP2	hyaluronan binding protein 2	11.0	Publications
20	NES	nestin	11.0	Publications
21	EIF2AK3	eukaryotic translation initiation factor 2-alpha kinase 3	11.0	Publications
22	PSIP1	PC4 and SFRS1 interacting protein 1	11.0	Publications
23	CTSK	cathepsin K	11.0	Publications
24	HMGA1	high mobility group AT-hook 1	11.0	Publications
25	HMGA2	high mobility group AT-hook 2	11.0	Publications
26	MMP14	matrix metallopeptidase 14 (membrane-inserted)	11.0	Publications
27	ENO2	enolase 2 (gamma, neuronal)	11.0	Publications
28	ETS1	v-ets erythroblastosis virus E26 oncogene homolog 1 (avian)	11.0	Publications
29	HTT	huntingtin	11.0	Publications
30	MMP3	matrix metallopeptidase 3 (stromelysin 1, progelatinase)	11.0	Publications
31	PDGFRA	platelet-derived growth factor receptor, alpha polypeptide	11.0	Publications
32	TGFBR2	transforming growth factor, beta receptor II (70/80kDa)	11.0	Publications
33	MMP1	matrix metallopeptidase 1 (interstitial collagenase)	11.0	Publications
34	ABL1	c-abl oncogene 1, non-receptor tyrosine kinase	11.0	Publications
35	MMP2	matrix metallopeptidase 2 (gelatinase A, 72kDa gelatinase, 72kDa type IV collagenase)	11.0	Publications
36	PTGS2	prostaglandin-endoperoxide synthase 2 (prostaglandin G/H synthase and cyclooxygenase)	11.0	Publications
37	TP53	tumor protein p53	11.0	Publications

Pathways related to dermatofibrosarcoma protuberans according to GeneDecks:

(show all 50)

id	Pathway	Score	Top Affiliating Genes
1	Breast Cancer Regulation by Stathmin136	10.2	PDGFB, PSIP1
2	Cytoskeleton remodeling_Role of PDGFs in cell migration41	10.2	PDGFRB, PDGFRA, PDGFB
3	Cytoskeleton remodeling Role of PDGFs in cell migration10	10.2	PDGFRB, PDGFRA, PDGFB
4	14-3-3 Induced Intracellular Signaling36	10.0	PSIP1, PDGFRB, PDGFRA, PDGFB
5	Rac1 Pathway36	10.0	PSIP1, PDGFRB, PDGFB
6	Pathway_PA16598005034	10.0	PDGFRB, PDGFRA, ETS1
7	DHA Signaling36	9.8	PSIP1, PDGFRB, PDGFRA, PDGFB, KIT
8	PKR Pathway36	9.7	PDGFRB, PDGFRA, PDGFB, TP53
9	Glioma20	9.7	TP53, PDGFB, PDGFRA, PDGFRB
10	ERK5 Signaling36	9.7	KIT, PDGFB, PDGFRA, PDGFRB, PSIP1
11	Melanoma20	9.7	PDGFRB, PDGFRA, PDGFB, TP53
12	Nanog in Mammalian ESC Pluripotency36	9.7	SOX2, PSIP1, PDGFRB, PDGFRA, PDGFB, KIT
13	PPAR Pathway36	9.7	PSIP1, PDGFRB, PDGFRA, PDGFB, KIT
14	eIF2 Pathway36	9.7	PSIP1, EIF2AK3, PDGFRB, PDGFRA, PDGFB, KIT
15	Akt Signaling36	9.7	PSIP1, PDGFRB, PDGFRA, PDGFB, TNFAIP6, KIT
16	Prostate cancer20	9.6	TP53, PDGFB, PDGFRA, PDGFRB
17	Nuclear Receptor Activation by Vitamin-A36	9.6	KIT, PDGFB, PDGFRA, PDGFRB, PSIP1
18	GSK3 Signaling36	9.6	PSIP1, EIF2AK3, PDGFRB, PDGFRA, PDGFB, KIT
19	Glioblastoma Multiforme36	9.5	TP53, PDGFB, PDGFRA, PDGFRB
20	Rho Family GTPases36	9.5	PSIP1, DES, PDGFRB, PDGFRA, PDGFB, COL1A1
21	ERK Signaling36	9.5	PSIP1, PDGFRB, PDGFRA, PDGFB, COL1A1, ETS1
22	Apoptotic Pathways in Synovial Fibroblasts36	9.4	PSIP1, PDGFRB, PDGFRA, PDGFB, TP53, KIT
23	p53 Mediated Apoptosis36	9.4	PSIP1, PDGFRB, PDGFRA, PDGFB, TP53, KIT
24	Mitochondrial Apoptosis36	9.4	PSIP1, PDGFRB, PDGFRA, PDGFB, TP53, KIT
25	Phospholipase-C Pathway36	9.4	KIT, COL1A1, PDGFB, PDGFRA, PDGFRB, PSIP1
26	Cellular Apoptosis Pathway36	9.4	PSIP1, PDGFRB, PDGFRA, PDGFB, TP53, KIT
27	PDGF Pathway36	9.3	PDGFRB, PDGFRA, PTGS2
28	JNK Pathway36	9.3	KIT, TP53, PDGFB, PDGFRA, PDGFRB, PSIP1
29	PTEN Pathway36	9.3	PSIP1, PDGFRB, PDGFRA, PDGFB, COL1A1, TP53
30	MAPK Signaling36	9.2	PSIP1, PDGFRB, PDGFRA, PDGFB, COL1A1, TP53
31	Tyrosine Kinases / Adaptors3	9.2	KIT, PDGFRA, PDGFRB, ABL1
32	p70S6K Signaling36	9.2	PSIP1, PDGFRB, PDGFRA, PDGFB, KIT
33	UPA-UPAR Pathway36	9.2	COL1A1, MMP3, MMP2, MMP11, MMP1
34	p38 Signaling36	9.2	PSIP1, PDGFRB, PDGFRA, PDGFB, TNFAIP6, TP53
35	Immune response_MIF-mediated glucocorticoid regulation41	9.2	PTGS2, ETS1, MMP1
36	Immune response MIF-mediated glucocorticoid regulation10	9.2	PTGS2, ETS1, MMP1
37	Activation of Matrix Metalloproteinases38	9.1	MMP3, MMP2, MMP14, MMP11, MMP1
38	Inhibition of Matrix Metalloproteinases36	9.1	MMP3, MMP2, MMP14, MMP11, MMP1
39	Metalloproteases in connective tissue degradation10	9.1	COL1A1, MMP3, MMP2, MMP14, MMP1
40	Cell adhesion_ECM remodeling41	9.1	COL1A1, MMP3, MMP2, MMP14, MMP1
41	Cell adhesion ECM remodeling10	9.1	COL1A1, MMP3, MMP2, MMP14, MMP1
42	Renin-Angiotensin Pathway36	9.1	PSIP1, PDGFRB, PDGFRA, PDGFB, PTGS2, KIT
43	Neuroscience3	8.9	PSIP1, PTGS2, HTT, ENO2, S100B
44	Telomerase Components in Cell Signaling36	8.9	PSIP1, ABL1, PDGFRB, PDGFRA, PDGFB, TP53
45	ILK Signaling36	8.7	PSIP1, PDGFRB, PDGFRA, PDGFB, COL1A1, PTGS2
46	Angiogenesis3	8.7	PDGFRB, PDGFRA, PTGS2, ETS1, MMP2
47	Molecular Mechanisms of Cancer36	8.3	SOX2, PSIP1, TGFBR2, ABL1, PDGFRA, PDGFB
48	Pathways in cancer20	7.7	TGFBR2, ABL1, PDGFRA, TP53, ETS1, MMP2
49	Colorectal Cancer Metastasis36	7.7	TGFBR2, PTGS2, TP53, MMP2, MMP14, MMP11
50	Pancreatic Adenocarcinoma36	6.7	KIT, PSIP1, TGFBR2, PDGFRB, PDGFRA, PDGFB

Compounds related to dermatofibrosarcoma protuberans according to GeneDecks:

(show top 50)    (show all 141)

id	Compound	Score	Top Affiliating Genes
1	egcg32	10.5	PDGFRB
2	orcein32	10.2	VIM, DES, S100B, ACTC1
3	mucicarmine32	10.0	S100A1, VIM, MUC1, S100B, ACTC1, DES
4	reticulin32	10.0	S100B, MUC1, DES, VIM, CD34, F13A1
5	toluidine32	10.0	VIM, DES, S100A1, ENO2, KIT
6	b 72332	9.9	ACTC1, VIM, MUC1, S100B, F13A1, DES
7	s 10032	9.8	VIM, MUC1, S100B, ENO2, F13A1, ACTC1
8	glucose32	9.5	ACTC1, DES, EIF2AK3, ETS1, S100B, NES
9	bromodeoxyuridine32	9.4	PDGFB, PDGFRB, MMP14, NES, ENO2, DES
10	12-o-tetradecanoylphorbol 13-acetate32	9.4	CD34, MMP11, MMP14, MMP2, MMP3
11	silver32	9.3	DES, PDGFB, F13A1, CD34, ENO2, S100A1
12	formaldehyde32 18	10.1	DES, ACTC1, ENO2, MMP1, S100B, VIM
13	imatinib32 34 9 9	12.0	S100B, COL1A1, PDGFB, PDGFRA, PDGFRB, ABL1
14	ilomastat32	9.0	MMP1, MMP3, MMP14, MMP2
15	glycogen32 18	10.0	ENO2, HTT, VIM, MUC1, S100A1, DES
16	chloramphenicol32 9 9	10.9	MUC1, S100B, MMP14, MMP3, HTT, ETS1
17	aspartate32	8.8	MMP2, S100B, CTSK, VIM, ABL1
18	marimastat32 42 9 9	11.8	MMP14, MMP11, MMP1, MMP2, MMP3
19	hematoxylin32	8.6	S100B, KIT, VIM, MUC1, S100A1, F13A1
20	1,10-phenanthroline32	8.6	MMP1, CTSK, MMP3, MMP2, MMP14
21	procollagen32	8.3	PDGFB, ACTC1, DES, COL1A1, F13A1, VIM
22	thymidine32 18	9.2	ETS1, S100B, COL1A1, PDGFB, PDGFRA, DES
23	iron32 18	9.1	S100B, DES, ACTC1, PTGS2, F13A1, HTT
24	hyaluronic acid32 18	9.1	HTT, ENO2, DES, MMP1, MUC1, VIM
25	tgf beta132	8.0	MMP2, TGFBR2, PDGFRB, PDGFRA, ETS1, MMP1
26	testosterone32 9 18 9	10.9	DES, PDGFRB, HTT, ENO2, APOD, MMP2
27	matrigel32	7.9	PDGFRB, ACTC1, NES, VIM, HMGA1, ETS1
28	progesterone32 42 9 18 9	11.9	KIT, NES, S100A1, S100B, CD34, MMP11
29	creatinine32	7.9	MMP2, ENO2, ACTC1, DES, APOD, MMP3
30	lactate32	7.8	MMP3, KIT, VIM, MMP1, MMP14, MMP2
31	proline32	7.8	MMP11, HTT, TP53, CTSK, P4HTM, ABL1
32	cysteine32	7.8	MMP1, S100B, MUC1, S100A1, HTT, TGFBR2
33	ribonucleic acid32	7.7	MMP1, KIT, MUC1, MMP14, MMP2, MMP3
34	actinomycin d32	7.6	TGFBR2, PTGS2, TP53, HMGA2, ETS1, MMP14
35	lipid32	7.5	NES, ABL1, PDGFB, PDGFRB, S100B, MUC1
36	nitric oxide32 9 18 9	10.4	MUC1, KIT, DES, PTGS2, PDGFB, EIF2AK3
37	alanine32	7.4	ENO2, MMP3, S100B, MMP14, TGFBR2, VIM
38	pd 98,05932	7.2	KIT, EIF2AK3, PDGFB, ETS1, ENO2, MMP3
39	oxygen32 18	8.2	P4HTM, MMP14, MMP11, PDGFB, MMP3, ETS1
40	dexamethasone32 42 34 9 9	11.2	S100B, MUC1, VIM, MMP3, ENO2, TNFAIP6
41	zinc32 18	8.1	MMP2, MMP3, COL1A1, MMP14, MMP11, MMP1
42	oligonucleotide32	6.7	ABL1, F13A1, TGFBR2, MMP14, PDGFRA, PDGFB
43	cycloheximide32	6.5	MMP2, KIT, TGFBR2, PDGFB, PTGS2, TP53
44	retinoic acid32 42 18	8.5	MMP11, MMP1, CD34, S100B, KIT, MMP3
45	estrogen32	6.5	PDGFRB, PDGFRA, PDGFB, COL1A1, HMGA1, F13A1
46	calcium32 9 18 9	9.0	TNFAIP6, ETS1, F13A1, HTT, MMP2, MMP14
47	paraffin32	5.7	TP53, PDGFB, PDGFRA, PDGFRB, ACTC1, HMGA2
48	tyrosine32	5.0	TGFBR2, ACTC1, EIF2AK3, ABL1, PDGFRB, PDGFRA
49	serine32	4.9	HABP2, DES, TGFBR2, EIF2AK3, ABL1, PDGFRB
50	vegf32	4.2	NES, DES, TGFBR2, ACTC1, ABL1, PDGFRB

Cellular components related to dermatofibrosarcoma protuberans according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	intrinsic to plasma membrane	GO:031226	10.0	PDGFRA, PDGFRB
2	senescence-associated heterochromatin focus	GO:035985	9.9	HMGA2, HMGA1
3	extracellular region	GO:005576	7.8	MUC1, HABP2, PDGFB, COL1A1, F13A1, APOD
4	cytoplasm	GO:005737	5.7	PDGFRA, PDGFRB, ABL1, EIF2AK3, ACTC1, SOX2

Biological processes related to dermatofibrosarcoma protuberans according to GeneDecks:

(show all 30)

id	Name	GO ID	Score	Top Affiliating Genes
1	paracrine signaling	GO:038001	10.6	PDGFB, CD34
2	metanephric glomerular mesangial cell proliferation involved in metanephros development	GO:072262	10.6	PDGFRB, PDGFB
3	positive regulation of metanephric mesenchymal cell migration by platelet-derived growth factor receptor-beta signaling pathway	GO:035793	10.4	PDGFRB, PDGFB
4	oncogene-induced senescence	GO:090402	10.3	HMGA2, HMGA1
5	metanephric glomerular capillary formation	GO:072277	10.2	PDGFRB, PDGFRA
6	positive regulation of cell proliferation by VEGF-activated platelet derived growth factor receptor signaling pathway	GO:038091	10.2	PDGFRB, PDGFRA
7	cardiac myofibril assembly	GO:055003	10.2	PDGFRA, PDGFRB, ACTC1
8	positive regulation of phospholipase C activity	GO:010863	10.2	KIT, PDGFRA, PDGFRB
9	platelet-derived growth factor receptor signaling pathway	GO:048008	10.2	PDGFB, PDGFRA, PDGFRB
10	senescence-associated heterochromatin focus assembly	GO:035986	10.2	HMGA2, HMGA1
11	cell chemotaxis	GO:060326	10.1	KIT, PDGFB, PDGFRA, PDGFRB
12	positive regulation of phosphatidylinositol 3-kinase activity	GO:043552	10.1	PDGFRB, PDGFRA, PDGFB, KIT
13	muscle filament sliding	GO:030049	10.1	VIM, ACTC1, DES
14	positive regulation of phosphatidylinositol 3-kinase cascade	GO:014068	10.1	PDGFRB, PDGFRA, PDGFB, KIT
15	positive regulation of cellular senescence	GO:2000774	10.0	HMGA2, HMGA1
16	intramembranous ossification	GO:001957	10.0	COL1A1, CTSK
17	positive regulation of reactive oxygen species metabolic process	GO:2000379	9.7	TP53, PDGFB, PDGFRB, TGFBR2
18	positive regulation of cell migration	GO:030335	9.6	PDGFRB, PDGFRA, PDGFB, COL1A1, ETS1, MMP14
19	peptidyl-tyrosine phosphorylation	GO:018108	9.6	ABL1, PDGFRB, PDGFRA, PDGFB, KIT
20	positive regulation of transforming growth factor beta production	GO:071636	9.5	PTGS2, CD34
21	collagen catabolic process	GO:030574	9.3	MMP3, MMP2, MMP11, MMP1
22	response to estradiol stimulus	GO:032355	9.2	PDGFB, PTGS2, ETS1, ENO2
23	base-excision repair	GO:006284	9.2	HMGA1, HMGA2, TP53
24	positive regulation of cell proliferation	GO:008284	9.1	TGFBR2, PDGFRB, PDGFRA, PDGFB, ETS1, KIT
25	extracellular matrix disassembly	GO:022617	9.1	MMP1, MMP11, MMP14, MMP2, MMP3
26	positive regulation of transcription, DNA-dependent	GO:045893	9.0	SOX2, PDGFB, COL1A1, TP53, HMGA2, HMGA1
27	extracellular matrix organization	GO:030198	8.9	COL1A1, MMP3, MMP2, MMP14, MMP11, MMP1
28	virus-host interaction	GO:019048	8.8	PSIP1, PDGFRA, TP53, HMGA1, MMP1, VIM
29	response to drug	GO:042493	8.7	NES, TGFBR2, ACTC1, PDGFB, PTGS2, ENO2
30	proteolysis	GO:006508	8.5	HABP2, CTSK, MMP3, MMP2, MMP14, MMP11

Molecular functions related to dermatofibrosarcoma protuberans according to GeneDecks:

(show all 9)

id	Name	GO ID	Score	Top Affiliating Genes
1	platelet-derived growth factor receptor binding	GO:005161	10.0	PDGFB, PDGFRA, PDGFRB
2	5-deoxyribose-5-phosphate lyase activity	GO:051575	10.0	HMGA1, HMGA2
3	vascular endothelial growth factor binding	GO:038085	10.0	PDGFRA, PDGFRB
4	platelet-derived growth factor binding	GO:048407	9.9	PDGFRB, PDGFRA, PDGFB, COL1A1
5	identical protein binding	GO:042802	9.3	EIF2AK3, COL1A1, TP53, S100B, S100A1, VIM
6	metalloendopeptidase activity	GO:004222	8.8	MMP3, MMP2, MMP14, MMP11, MMP1
7	transcription factor binding	GO:008134	8.7	TP53, HMGA2, HMGA1, ETS1, CD34
8	protein homodimerization activity	GO:042803	8.4	PDGFRA, PDGFB, PTGS2, ENO2, S100B, S100A1
9	protein binding	GO:005515	4.7	KIT, COL1A1, PDGFB, PDGFRA, PDGFRB, ABL1