DFSP
MCID: DRM014
MIFTS: 61

Dermatofibrosarcoma Protuberans (DFSP) malady

Categories: Genetic diseases, Rare diseases, Skin diseases, Cancer diseases

Aliases & Classifications for Dermatofibrosarcoma Protuberans

Aliases & Descriptions for Dermatofibrosarcoma Protuberans:

Name: Dermatofibrosarcoma Protuberans 54 12 50 24 25 56 29 52 14 69
Dermatofibrosarcoma 25 52 42 69
Dfsp 50 25 56
Metastatic Dermatofibrosarcoma Protuberans 50 69
Giant Cell Fibroblastoma 50 69
Familial Dermatofibrosarcoma Protuberans 50
Darier-Hoffmann Tumor 25
Darier-Ferrand Tumor 25

Characteristics:

Orphanet epidemiological data:

56
dermatofibrosarcoma protuberans
Inheritance: Not applicable; Prevalence: 1-5/10000 (Europe); Age of onset: All ages; Age of death: adult;

Classifications:

Orphanet: 56  
Rare skin diseases


External Ids:

OMIM 54 607907
Disease Ontology 12 DOID:3507
MeSH 42 D018223
Orphanet 56 ORPHA31112
MESH via Orphanet 43 C538219
UMLS via Orphanet 70 C0392784
ICD10 via Orphanet 34 C49.9
UMLS 69 C0206647

Summaries for Dermatofibrosarcoma Protuberans

NIH Rare Diseases : 50 dermatofibrosarcoma protuberans is an uncommon cancer in which tumors arise in the deeper layers of skin. the tumor usually starts as a small, firm patch of skin; it may be purplish, reddish, or flesh-colored. it is commonly found on the torso, usually in the shoulder and chest area. the tumor typically grows slowly but has a tendency to recur after being removed. it rarely spreads to other parts of the body. the cause of dfsp is unknown, but injury to the affected skin may be a predisposing factor. treatment usually involves surgically removing the tumor. if the tumor is unable to be removed completely, additional therapy may be needed. regular follow-up is important to monitor for recurrence. last updated: 2/9/2016

MalaCards based summary : Dermatofibrosarcoma Protuberans, also known as dermatofibrosarcoma, is related to gastrointestinal stromal tumor and bednar tumor, and has symptoms including subcutaneous nodule, skin ulcer and neoplasm of the skin. An important gene associated with Dermatofibrosarcoma Protuberans is PDGFB (Platelet Derived Growth Factor Subunit B), and among its related pathways/superpathways are 4-1BB Pathway and Immune response IL-23 signaling pathway. The drugs Doxorubicin and Ifosfamide have been mentioned in the context of this disorder. Affiliated tissues include skin, breast and lung, and related phenotypes are homeostasis/metabolism and cardiovascular system

Genetics Home Reference : 25 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.

OMIM : 54 Dermatofibrosarcoma protuberans (DFSP) is an uncommon, locally aggressive, but rarely metastasizing tumor of the deep... (607907) more...

Disease Ontology : 12 A fibrosarcoma that is located in the dermis laryer of the skin and that begins as a hard nodule and grows slowly.

Wikipedia : 71 Dermatofibrosarcoma protuberans (DFSP) is a very rare tumor. It is a rare neoplasm of the dermis layer... more...

Related Diseases for Dermatofibrosarcoma Protuberans

Diseases related to Dermatofibrosarcoma Protuberans via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 179)
id Related Disease Score Top Affiliating Genes
1 gastrointestinal stromal tumor 28.9 ACTC1 CD34 DES PDGFRA PDGFRB S100B
2 bednar tumor 12.0
3 myxofibrosarcoma 11.4
4 rohhad 10.3 COL1A1 PDGFB
5 typical urticaria pigmentosa 10.3 F13A1 S100B
6 lupus - neurological sequelae 10.3 S100B VIM
7 sarcoma 10.3
8 dystrophic epidermolysis bullosa, nails only 10.3 ACTC1 F13A1
9 inflammatory linear verrucous epidermal nevus 10.3 DES VIM
10 lymphomatoid papulosis 10.3 ACTC1 VIM
11 nutritional deficiency disease 10.3 CD34 VIM
12 secondary adrenal insufficiency 10.2 ACTC1 F13A1
13 fibrosarcoma 10.2
14 lymphomatous thyroiditis 10.2 DES VIM
15 postgerminal center chronic lymphocytic leukemia/small lymphocytic lymphoma 10.2 F13A1 S100B VIM
16 angiofollicular lymph hyperplasia 10.2 ACTC1 NES
17 kosaki overgrowth syndrome 10.2 ACTC1 PDGFRB
18 riboflavin transporter deficiency neuronopathy 10.2 PDGFB PDGFRB
19 acute generalized exanthematous pustulosis 10.2 ACTC1 S100B
20 histiocytoma 10.2
21 fibrous histiocytoma 10.2
22 pathologic nystagmus 10.2 CD34 F13A1 S100B
23 chest wall parachordoma 10.2 ACTC1 CD34 VIM
24 carbon monoxide-induced parkinsonism 10.2 ACTC1 DES
25 intrahepatic bile duct cystadenoma 10.2 DES S100B
26 testis sarcoma 10.2 CD34 DES
27 ritscher-schinzel syndrome 1 10.2 CD34 F13A1
28 myeloid and lymphoid neoplasms associated with fgfr1 abnormalities 10.2 PDGFRA PDGFRB
29 jewett-marshall bladder cancer 10.2 CD34 DES VIM
30 male reproductive system disease 10.2 DES NES VIM
31 ovarian epithelial cancer 10.2 NES S100B VIM
32 biliary papillomatosis 10.2 DES S100B VIM
33 jejunal neuroendocrine tumor 10.2 S100B VIM
34 vulvar squamous papilloma 10.2 DES S100B VIM
35 fibroma 10.2
36 chiasmal syndrome 10.2 ACTC1 S100B
37 epimetaphyseal dysplasia cataract 10.2 NES S100B VIM
38 invasive mole 10.2 PDGFRA S100B
39 benign schwannoma 10.2 COL1A1 PDGFB S100B VIM
40 vagina leiomyosarcoma 10.2 ACTC1 DES
41 13q12.3 microdeletion syndrome 10.2 ACTC1 DES VIM
42 cortical blindness 10.2 F13A1 VIM
43 autosomal dominant proximal renal tubular acidosis 10.2 PDGFRA PDGFRB
44 hypogonadotropic hypogonadism-retinitis pigmentosa syndrome 10.2 ACTC1 S100B VIM
45 arteriosclerosis obliterans 10.1 CD34 PDGFRB TP53
46 scn8a-related epilepsy with encephalopathy 10.1 F13A1 HABP2
47 desmoid tumor 10.1 ACTC1 S100B VIM
48 osteomyelitis 10.1 ACTC1 DES VIM
49 episodic ataxia 10.1 ACTC1 CD34 DES
50 intestinal benign neoplasm 10.1 PDGFB SERPINA3 VIM

Graphical network of the top 20 diseases related to Dermatofibrosarcoma Protuberans:



Diseases related to Dermatofibrosarcoma Protuberans

Symptoms & Phenotypes for Dermatofibrosarcoma Protuberans

Clinical features from OMIM:

607907

Human phenotypes related to Dermatofibrosarcoma Protuberans:

56 32 (show all 6)
id Description HPO Frequency Orphanet Frequency HPO Source Accession
1 subcutaneous nodule 56 32 Very frequent (99-80%) HP:0001482
2 skin ulcer 56 32 Frequent (79-30%) HP:0200042
3 neoplasm of the skin 56 32 Very frequent (99-80%) HP:0008069
4 erythema 56 32 Very frequent (99-80%) HP:0010783
5 thickened skin 56 32 Very frequent (99-80%) HP:0001072
6 fibrosarcoma 56 32 Very frequent (99-80%) HP:0100244

MGI Mouse Phenotypes related to Dermatofibrosarcoma Protuberans:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 homeostasis/metabolism MP:0005376 10.17 ACTC1 APOD CD163 CD34 COL1A1 DES
2 cardiovascular system MP:0005385 10.14 DES F13A1 PDGFB PDGFRA PDGFRB TP53
3 hematopoietic system MP:0005397 10.02 COL1A1 F13A1 PDGFB PDGFRA PDGFRB TP53
4 immune system MP:0005387 9.91 CD163 CD34 COL1A1 HABP2 PDGFB PDGFRA
5 integument MP:0010771 9.7 CD34 COL1A1 PDGFB PDGFRA PDGFRB S100B
6 mortality/aging MP:0010768 9.7 ACTC1 APOD COL1A1 DES F13A1 HABP2
7 muscle MP:0005369 9.28 ACTC1 CD163 COL1A1 DES PDGFB PDGFRA

Drugs & Therapeutics for Dermatofibrosarcoma Protuberans

Drugs for Dermatofibrosarcoma Protuberans (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 28)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Doxorubicin Approved, Investigational Phase 3,Phase 1 23214-92-8 31703
2
Ifosfamide Approved Phase 3 3778-73-2 3690
3
Mechlorethamine Approved Phase 3 51-75-2 4033
4
Doxil Approved June 1999 Phase 3,Phase 1 31703
5 Alkylating Agents Phase 3,Phase 1
6 Anti-Bacterial Agents Phase 3,Phase 1
7 Antibiotics, Antitubercular Phase 3,Phase 1
8 Antineoplastic Agents, Alkylating Phase 3,Phase 1
9 Isophosphamide mustard Phase 3
10 Topoisomerase Inhibitors Phase 3,Phase 1
11
Sunitinib Approved, Investigational Phase 2 341031-54-7, 557795-19-4 5329102
12
Paclitaxel Approved, Vet_approved Phase 1, Phase 2 33069-62-4 36314
13 Imatinib Mesylate Phase 2,Phase 1 123596
14 Protein Kinase Inhibitors Phase 2,Phase 1
15 Angiogenesis Inhibitors Phase 2
16 Angiogenesis Modulating Agents Phase 2
17 Immunosuppressive Agents Phase 2,Phase 1
18
saracatinib Phase 2
19 Albumin-Bound Paclitaxel Phase 1, Phase 2
20 Antimitotic Agents Phase 1, Phase 2
21 Antineoplastic Agents, Phytogenic Phase 1, Phase 2
22 taxane Phase 1, Phase 2
23 tyrosine Nutraceutical Phase 1, Phase 2
24
Cyclophosphamide Approved, Investigational Phase 1 50-18-0, 6055-19-2 2907
25 Antibodies Phase 1
26 Antibodies, Monoclonal Phase 1
27 Immunoglobulins Phase 1
28 Antirheumatic Agents Phase 1

Interventional clinical trials:

(show all 20)
id Name Status NCT ID Phase
1 Observation, Radiation Therapy, Combination Chemotherapy, and/or Surgery in Treating Young Patients With Soft Tissue Sarcoma Unknown status NCT00346164 Phase 3
2 Ifosfamide and Doxorubicin, Radiation Therapy, and/or Surgery in Treating Young Patients With Localized Soft Tissue Sarcoma Unknown status NCT00334854 Phase 3
3 Imatinib Mesylate (Gleevec) in the Treatment of Systemic Sclerosis Unknown status NCT00555581 Phase 2
4 Neoadjuvant Imatinib in Dermatofibrosarcoma Protuberans Completed NCT00243191 Phase 2
5 Imatinib in Dermatofibrosarcoma Protuberans (DFSP) Completed NCT00122473 Phase 1, Phase 2
6 Imatinib Mesylate in Treating Patients With Locally Recurrent or Metastatic Dermatofibrosarcoma Protuberans Completed NCT00084630 Phase 2
7 Imatinib Mesylate in Treating Patients With Locally Advanced or Metastatic Dermatofibrosarcoma Protuberans or Giant Cell Fibroblastoma Completed NCT00085475 Phase 2
8 Imatinib Mesylate in Patients With Various Types of Malignancies Involving Activated Tyrosine Kinase Enzymes Completed NCT00171912 Phase 2
9 Sunitinib in Treating Patients With Metastatic, Locally Advanced, or Locally Recurrent Sarcomas Completed NCT00474994 Phase 2
10 AZD0530 in Treating Patients With Recurrent Locally Advanced or Metastatic Soft Tissue Sarcoma Completed NCT00659360 Phase 2
11 Vismodegib and Gamma-Secretase/Notch Signalling Pathway Inhibitor RO4929097 in Treating Patients With Advanced or Metastatic Sarcoma Completed NCT01154452 Phase 1, Phase 2
12 Phase II Study of Imatinib Mesylate in Patients With Life Threatening Malignant Rare Diseases Completed NCT00154388 Phase 2
13 To Find a Safe Dose and Show Early Clinical Activity of Weekly Nab-paclitaxel in Pediatric Patients With Recurrent/ Refractory Solid Tumors Recruiting NCT01962103 Phase 1, Phase 2
14 Phase II Pazopanib Study in Advanced Dermatofibrosarcomas Terminated NCT01059656 Phase 2
15 Intensity-Modulated Radiation Therapy in Treating Patients Undergoing Surgery for Stage IB, Stage II, or Stage III Soft Tissue Sarcoma Terminated NCT00740597 Phase 2
16 Cixutumumab and Doxorubicin Hydrochloride in Treating Patients With Unresectable, Locally Advanced, or Metastatic Soft Tissue Sarcoma Completed NCT00720174 Phase 1
17 Imatinib Mesylate And Cyclophosphamide In Metronomic Administration: Dose Escalation Study Of Imatinib Mesylate Completed NCT01046487 Phase 1
18 Clinical Outcome of Pediatric Dermatofibrosarcoma Protuberans Unknown status NCT00173355
19 Spanish Registry of Mohs Surgery Recruiting NCT02310503
20 Glutamine in Preventing Oral Mucositis in Patients Receiving Chemotherapy for Sarcoma Withdrawn NCT00334984

Search NIH Clinical Center for Dermatofibrosarcoma Protuberans

Cochrane evidence based reviews: dermatofibrosarcoma

Genetic Tests for Dermatofibrosarcoma Protuberans

Genetic tests related to Dermatofibrosarcoma Protuberans:

id Genetic test Affiliating Genes
1 Dermatofibrosarcoma Protuberans 29 24 PDGFB

Anatomical Context for Dermatofibrosarcoma Protuberans

MalaCards organs/tissues related to Dermatofibrosarcoma Protuberans:

39
Skin, Breast, Lung, Bone, Lymph Node, Endothelial, Pancreas

The Foundational Model of Anatomy Ontology organs/tissues related to Dermatofibrosarcoma Protuberans:

18
The Dermis Laryer Of The Skin

Publications for Dermatofibrosarcoma Protuberans

Articles related to Dermatofibrosarcoma Protuberans:

(show top 50) (show all 644)
id Title Authors Year
1
A single-institutional review of 68 patients with dermatofibrosarcoma protuberans: wide re-excision after inadequate previous surgery results in a high rate of local control. ( 28056985 )
2017
2
When Immunohistochemistry Deceives Us: The Pitfalls of CD34 and Factor XIIIa Stains in Dermatofibroma and Dermatofibrosarcoma Protuberans. ( 28270311 )
2017
3
Incidence and Outcomes of Dermatofibrosarcoma Protuberans in the US Pediatric Population. ( 27922973 )
2017
4
Association of patient demographic characteristics with Dermatofibrosarcoma Protuberans tumor size at diagnosis in the National Cancer Data Base. ( 28144941 )
2017
5
Breast and pleuropulmonary metastasis of multirecurrent scalp dermatofibrosarcoma protuberans: a case report. ( 28388964 )
2017
6
Dermatofibrosarcoma Protuberans: An Immunomarker Study of 57 Cases That Included Putative Mesenchymal Stem Cell Markers. ( 28362700 )
2017
7
A Comparison of Mohs Micrographic Surgery and Wide Local Excision for Treatment of Dermatofibrosarcoma Protuberans With Long-Term Follow-up: The Mayo Clinic Experience. ( 27749444 )
2017
8
Clinical Features and Treatment of Dermatofibrosarcoma Protuberans Affecting the Vulva: A Literature Review. ( 28323651 )
2017
9
Dermatofibrosarcoma protuberans of the scalp: therapeutic challenges. ( 28513108 )
2017
10
Dermatofibrosarcoma protuberans: when the age makes the difference. ( 28509521 )
2017
11
Subcutaneous dermatofibrosarcoma protuberans, a rare subtype with predilection for the head: A retrospective series of 18 cases. ( 28420485 )
2017
12
Long-term results of treatment of advanced dermatofibrosarcoma protuberans (DFSP) with imatinib mesylate - The impact of fibrosarcomatous transformation. ( 28365129 )
2017
13
Toluidine Blue Stain of Dermatofibrosarcoma Protuberans: Highlighting Its Use in Mohs. ( 28346254 )
2017
14
Programmed death-ligand 1 expression is associated with fibrosarcomatous transformation of dermatofibrosarcoma protuberans. ( 28515919 )
2017
15
Sclerosing Dermatofibrosarcoma Protuberans Shows Significant Overlap With Sclerotic Fibroma in Both Routine and Immunohistochemical Analysis: A Potential Diagnostic Pitfall. ( 28134725 )
2017
16
Operative management of dermatofibrosarcoma protuberans of the breast. ( 28154469 )
2017
17
Application of COL1A1-PDGFB fusion gene detection by fluorescence inA situ hybridization in biopsy tissue of dermatofibrosarcoma protuberans. ( 28150334 )
2017
18
Dermoscopy of uncommon variants of dermatofibrosarcoma protuberans. ( 28191686 )
2017
19
Myxoid Dermatofibrosarcoma Protuberans of the Abdomen. ( 26816559 )
2016
20
Dermatofibrosarcoma Protuberans-Like Tumor With COL1A1 Copy Number Gain in the Absence of t(17;22). ( 27984233 )
2016
21
Dermatofibrosarcoma Protuberans: Insights into a Rare Soft Tissue Tumor. ( 27195182 )
2016
22
A Case of Dermatofibrosarcoma Protuberans Arising in Subcutaneous Tissue of the Breast. ( 27346162 )
2016
23
Adaptive immunity in fibrosarcomatous dermatofibrosarcoma protuberans and response to imatinib treatment. ( 27608549 )
2016
24
Effectiveness of Imatinib Mesylate Treatment in a Patient with Dermatofibrosarcoma Protuberans with Pulmonary and Pancreatic Metastases. ( 27580559 )
2016
25
Dermatofibrosarcoma protuberans: a case report and review of the literature. ( 27895450 )
2016
26
Hormone receptor expression in patients with dermatofibrosarcoma protuberans. ( 27542587 )
2016
27
Case of pigmented dermatofibrosarcoma protuberans with atrophic change. ( 27029004 )
2016
28
Paraffin-embedded micrographic surgery for the treatment of dermatofibrosarcoma protuberans: Analysis of 33 patients. ( 27643548 )
2016
29
Dermatofibrosarcoma protuberans: pathology, genetics, and potential therapeutic strategies. ( 27806849 )
2016
30
A rare case of dermatofibrosarcoma protuberans of the forefoot. ( 27257461 )
2016
31
Nodular Fasciitis Complicating a Staged Surgical Excision of Dermatofibrosarcoma Protuberans. ( 28018683 )
2016
32
Prognostic Factors of Survival in Dermatofibrosarcoma Protuberans. ( 27973651 )
2016
33
Dermatofibrosarcoma protuberans-a rare neoplasm. ( 27190778 )
2016
34
Bednar Tumor (Pigmented Dermatofibrosarcoma Protuberans). ( 26945477 )
2016
35
Operative management of dermatofibrosarcoma protuberans of the breast. ( 27365884 )
2016
36
Prognostic Factors, Treatment, and Survival in Dermatofibrosarcoma Protuberans. ( 27262160 )
2016
37
Histologic Features Associated with Deep Invasion in Dermatofibrosarcoma Protuberans. ( 26944448 )
2016
38
InA vivo characterization of recurrent dermatofibrosarcoma protuberans by dermoscopy and reflectance confocal microscopy. ( 27745647 )
2016
39
Imaging Features of Breast Dermatofibrosarcoma Protuberans in Various Modalities Including FDG-PET CT. ( 27703659 )
2016
40
Congenital atrophic dermatofibrosarcoma protuberans detected by COL1A1-PDGFB rearrangement. ( 26932148 )
2016
41
Dermatofibrosarcoma Protuberans: Wide Local Excision Versus Mohs Micrographic Surgery. ( 27591501 )
2016
42
Improving precision of resection by pre-surgery inspections with contrast-enhanced ultrasound for dermatofibrosarcoma protuberans. ( 27572421 )
2016
43
Evolution of Dermatofibrosarcoma Protuberans to DFSP-Derived Fibrosarcoma: An Event Marked by Epithelial-Mesenchymal Transition-like Process and 22q Loss. ( 27256159 )
2016
44
Fibrosarcomatous change in the background of dermatofibrosarcoma protuberans in male breast: Study of a rare case and review of the entity. ( 27134483 )
2016
45
Scalp Dermatofibrosarcoma Protuberans: A Potential Diagnostic Pitfall. ( 26967087 )
2016
46
Clinicopathological features of dermatofibrosarcoma protuberans. ( 26870263 )
2016
47
Medallion-Like Dermal Dendrocytic Hamartoma, Dermatofibrosarcoma Protuberans, and Adenosine Deaminase-Deficient Severe Combined Immunodeficiency. ( 27176810 )
2016
48
Pazopanib induced a partial response in a patient with metastatic fibrosarcomatous dermatofibrosarcoma protuberans without genetic translocations resistant to mesna, doxorubicin, ifosfamide and dacarbazine chemotherapy and gemcitabine-docetaxel chemotherapy. ( 27988943 )
2016
49
Pedunculated Nodules as a Variant of Dermatofibrosarcoma Protuberans. ( 27746644 )
2016
50
The efficacy of adjuvant radiotherapy in dermatofibrosarcoma protuberans: a systemic review and meta-analysis. ( 26879523 )
2016

Variations for Dermatofibrosarcoma Protuberans

ClinVar genetic disease variations for Dermatofibrosarcoma Protuberans:

6
id Gene Variation Type Significance SNP ID Assembly Location
1 PDGFB PDGFB, PDGFB/COL1A1 FUSION undetermined variant Pathogenic

Cosmic variations for Dermatofibrosarcoma Protuberans:

9
id Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA Conf
1 COSM575 NRAS soft tissue,skin,sarcoma,NS c.38G>C p.G13A 8
2 COSM584 NRAS skin,shoulder,benign melanocytic nevus,congenital c.182A>G p.Q61R 6
3 COSM476 BRAF skin,trunk,benign melanocytic nevus,congenital c.1799T>A p.V600E 6
4 COSM28757 GNAQ soft tissue,chest,haemangioma,congenital c.626A>T p.Q209L 4

Expression for Dermatofibrosarcoma Protuberans

Search GEO for disease gene expression data for Dermatofibrosarcoma Protuberans.

Pathways for Dermatofibrosarcoma Protuberans

Pathways related to Dermatofibrosarcoma Protuberans according to GeneCards Suite gene sharing:

(show all 15)
id Super pathways Score Top Affiliating Genes
1
Show member pathways
12.45 PDGFB PDGFRA PDGFRB TP53
2
Show member pathways
12.37 PDGFB PDGFRA PDGFRB TP53
3 12.34 COL1A1 PDGFB PDGFRA PDGFRB TP53
4 12.2 PDGFB PDGFRA PDGFRB TP53 VIM
5 12.19 PDGFB PDGFRA PDGFRB TP53
6 11.8 NES PDGFB PDGFRB VIM
7 11.76 F13A1 TP53 VIM
8
Show member pathways
11.72 DES NES VIM
9 11.65 CD34 DES PDGFB
10 11.6 PDGFB PDGFRA PDGFRB TP53
11 11.49 ACTC1 DES VIM
12 11.42 PDGFRA PDGFRB TP53
13 11.26 NES PDGFB PDGFRA S100B VIM
14 10.69 PDGFB PDGFRA PDGFRB
15 10.07 COL1A1 PDGFB PDGFRA PDGFRB

GO Terms for Dermatofibrosarcoma Protuberans

Cellular components related to Dermatofibrosarcoma Protuberans according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 9.7 ACTC1 APOD COL1A1 HABP2 PDGFB S100B
2 extracellular matrix GO:0031012 9.56 COL1A1 NES PDGFB VIM
3 extracellular region GO:0005576 9.28 APOD CD163 CD34 COL1A1 F13A1 HABP2
4 platelet alpha granule lumen GO:0031093 9.13 F13A1 PDGFB SERPINA3
5 cytoplasm GO:0005737 10.1 ACTC1 CD34 COL1A1 DES F13A1 NES

Biological processes related to Dermatofibrosarcoma Protuberans according to GeneCards Suite gene sharing:

(show all 29)
id Name GO ID Score Top Affiliating Genes
1 positive regulation of gene expression GO:0010628 9.89 ACTC1 CD34 PDGFB TP53 VIM
2 positive regulation of cell migration GO:0030335 9.84 COL1A1 PDGFB PDGFRA PDGFRB
3 platelet degranulation GO:0002576 9.82 F13A1 PDGFB SERPINA3
4 phosphatidylinositol phosphorylation GO:0046854 9.8 PDGFB PDGFRA PDGFRB
5 wound healing GO:0042060 9.79 COL1A1 PDGFRA PDGFRB
6 regulation of phosphatidylinositol 3-kinase signaling GO:0014066 9.77 PDGFB PDGFRA PDGFRB
7 cell chemotaxis GO:0060326 9.73 PDGFB PDGFRA PDGFRB
8 positive regulation of phosphatidylinositol 3-kinase signaling GO:0014068 9.72 PDGFB PDGFRA PDGFRB
9 positive regulation of fibroblast proliferation GO:0048146 9.69 PDGFB PDGFRA PDGFRB
10 muscle filament sliding GO:0030049 9.65 ACTC1 DES VIM
11 intermediate filament organization GO:0045109 9.64 DES VIM
12 response to hyperoxia GO:0055093 9.63 COL1A1 PDGFRB
13 positive regulation of calcium ion import GO:0090280 9.63 PDGFB PDGFRB
14 positive regulation of DNA biosynthetic process GO:2000573 9.62 PDGFB PDGFRB
15 positive regulation of chemotaxis GO:0050921 9.61 PDGFB PDGFRB
16 stem cell proliferation GO:0072089 9.59 CD34 NES
17 positive regulation of phospholipase C activity GO:0010863 9.58 PDGFRA PDGFRB
18 negative regulation of platelet activation GO:0010544 9.58 PDGFB PDGFRA
19 retina vasculature development in camera-type eye GO:0061298 9.57 PDGFRA PDGFRB
20 positive regulation of reactive oxygen species metabolic process GO:2000379 9.54 PDGFB PDGFRB TP53
21 paracrine signaling GO:0038001 9.52 CD34 PDGFB
22 positive regulation of phosphatidylinositol 3-kinase activity GO:0043552 9.5 PDGFB PDGFRA PDGFRB
23 positive regulation of metanephric mesenchymal cell migration by platelet-derived growth factor receptor-beta signaling pathway GO:0035793 9.46 PDGFB PDGFRB
24 phosphatidylinositol-mediated signaling GO:0048015 9.46 PDGFB PDGFRA PDGFRB TP53
25 positive regulation of cell proliferation by VEGF-activated platelet derived growth factor receptor signaling pathway GO:0038091 9.43 PDGFRA PDGFRB
26 platelet-derived growth factor receptor signaling pathway GO:0048008 9.43 PDGFB PDGFRA PDGFRB
27 metanephric glomerular capillary formation GO:0072277 9.4 PDGFRA PDGFRB
28 cardiac myofibril assembly GO:0055003 9.13 ACTC1 PDGFRA PDGFRB
29 negative regulation of platelet-derived growth factor receptor-beta signaling pathway GO:2000587 8.8 PDGFB PDGFRA PDGFRB

Molecular functions related to Dermatofibrosarcoma Protuberans according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 9.73 COL1A1 DES PDGFB S100B TP53 VIM
2 Ras guanyl-nucleotide exchange factor activity GO:0005088 9.54 PDGFB PDGFRA PDGFRB
3 phosphatidylinositol-4,5-bisphosphate 3-kinase activity GO:0046934 9.5 PDGFB PDGFRA PDGFRB
4 vascular endothelial growth factor binding GO:0038085 9.16 PDGFRA PDGFRB
5 platelet-derived growth factor receptor binding GO:0005161 9.13 PDGFB PDGFRA PDGFRB
6 platelet-derived growth factor binding GO:0048407 8.92 COL1A1 PDGFB PDGFRA PDGFRB

Sources for Dermatofibrosarcoma Protuberans

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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