MCID: DRM014
MIFTS: 63

Dermatofibrosarcoma Protuberans

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

Aliases & Classifications for Dermatofibrosarcoma Protuberans

MalaCards integrated aliases for Dermatofibrosarcoma Protuberans:

Name: Dermatofibrosarcoma Protuberans 53 12 49 24 55 28 51 14 69
Dermatofibrosarcoma 24 51 41 69
Dfsp 53 49 24 55
Giant Cell Fibroblastoma 53 49 69
Metastatic Dermatofibrosarcoma Protuberans 49 69
Familial Dermatofibrosarcoma Protuberans 49
Darier-Hoffmann Tumor 24
Darier-Ferrand Tumor 24

Characteristics:

Orphanet epidemiological data:

55
dermatofibrosarcoma protuberans
Inheritance: Not applicable; Prevalence: 1-5/10000 (Europe); Age of onset: All ages; Age of death: adult;

Classifications:

Orphanet: 55  
Rare skin diseases


External Ids:

OMIM 53 607907
Disease Ontology 12 DOID:3507
MeSH 41 D018223
Orphanet 55 ORPHA31112
UMLS via Orphanet 70 C0392784
MESH via Orphanet 42 C538219
ICD10 via Orphanet 33 C49.9

Summaries for Dermatofibrosarcoma Protuberans

NIH Rare Diseases : 49 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 bednar tumor and myxofibrosarcoma, 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 PI3K-Akt signaling pathway and 4-1BB Pathway. The drugs Doxorubicin and Ifosfamide have been mentioned in the context of this disorder. Affiliated tissues include skin, lung and bone, and related phenotypes are cardiovascular system and homeostasis/metabolism

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.

Genetics Home Reference : 24 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 : 53 Dermatofibrosarcoma protuberans (DFSP) is an uncommon, locally aggressive, but rarely metastasizing tumor of the deep dermis and subcutaneous tissue. It typically presents during early or middle adult life and is most frequently located on the trunk and proximal extremities (Sandberg et al., 2003). (607907)

Wikipedia : 72 Dermatofibrosarcoma protuberans (DFSP) is a rare tumor. It is a rare neoplasm of the dermis layer of the... 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 195)
# Related Disease Score Top Affiliating Genes
1 bednar tumor 33.5 COL1A1 PDGFB S100B VIM
2 myxofibrosarcoma 32.8 DES S100B
3 ring chromosome 4 30.8 COL1A1 PDGFB
4 malignant giant cell tumor of soft parts 30.3 ACTC1 S100B
5 spindle cell lipoma 30.3 ACTC1 DES VIM
6 adult fibrosarcoma 30.1 COL1A1 PDGFB
7 cutaneous leiomyosarcoma 30.1 ACTC1 DES VIM
8 fasciitis 30.0 ACTC1 DES VIM
9 leiomyoma 30.0 ACTC1 CD34 DES
10 giant cell tumor 29.9 DES SERPINA3 VIM
11 histiocytoma 29.8 CD163 SERPINA3 TP53
12 malignant giant cell tumor 29.5 ACTC1 S100B TP53
13 hemangiopericytoma, malignant 29.4 ACTC1 DES F13A1 S100B VIM
14 fibrous histiocytoma 29.2 ACTC1 CD163 DES F13A1 SERPINA3 VIM
15 glomus tumor 29.2 ACTC1 DES TP53 VIM
16 meningioma, familial 28.9 CD34 PDGFB PDGFRB S100B TP53 VIM
17 leiomyosarcoma 27.8 ACTC1 DES PDGFRA PDGFRB S100B TP53
18 gastrointestinal stromal tumor 27.4 ACTC1 CD34 DES NES PDGFRA PDGFRB
19 undifferentiated pleomorphic sarcoma 11.2
20 primary familial brain calcification 10.5 PDGFB PDGFRB
21 sternum cancer 10.5 CD34 VIM
22 generalized eruptive histiocytosis 10.5 F13A1 S100B
23 multicentric reticulohistiocytosis 10.5 F13A1 VIM
24 basal ganglia calcification, idiopathic, 1 10.5 PDGFB PDGFRB
25 erythema elevatum diutinum 10.5 CD34 F13A1
26 hydromyelia 10.5 S100B VIM
27 granuloma annulare 10.5 F13A1 VIM
28 skin glomus tumor 10.4 DES VIM
29 progressive nodular histiocytosis 10.4 ACTC1 F13A1
30 infantile digital fibromatosis 10.4 DES VIM
31 primary hepatic neuroendocrine carcinoma 10.4 S100B VIM
32 lymphangiomatosis 10.4 DES VIM
33 scleromyxedema 10.4 ACTC1 F13A1
34 sarcoma 10.4
35 gallbladder sarcoma 10.4 CD34 DES
36 lymphangiectasis 10.4 ACTC1 VIM
37 chordoid meningioma 10.4 S100B VIM
38 primitive neuroectodermal tumor of the cervix uteri 10.4 DES VIM
39 myofibromatosis, infantile, 1 10.4 ACTC1 PDGFRB
40 anaplastic ganglioglioma 10.4 ACTC1 NES
41 epulis 10.4 DES VIM
42 eccrine porocarcinoma 10.4 S100B VIM
43 biphasic synovial sarcoma 10.4 DES VIM
44 kidney clear cell sarcoma 10.3 DES NES
45 fibrosarcoma 10.3
46 dysembryoplastic neuroepithelial tumor 10.3 NES S100B
47 malignant triton tumor 10.3 DES S100B
48 fibrous meningioma 10.3 F13A1 S100B VIM
49 mixed liposarcoma 10.3 CD34 PDGFB
50 adult xanthogranuloma 10.3 CD163 CD34

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:

55 31 (show all 6)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 subcutaneous nodule 55 31 hallmark (90%) Very frequent (99-80%) HP:0001482
2 skin ulcer 55 31 frequent (33%) Frequent (79-30%) HP:0200042
3 neoplasm of the skin 55 31 hallmark (90%) Very frequent (99-80%) HP:0008069
4 erythema 55 31 hallmark (90%) Very frequent (99-80%) HP:0010783
5 thickened skin 55 31 hallmark (90%) Very frequent (99-80%) HP:0001072
6 fibrosarcoma 55 31 hallmark (90%) Very frequent (99-80%) HP:0100244

MGI Mouse Phenotypes related to Dermatofibrosarcoma Protuberans:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 10.1 ACTC1 APOD CD163 COL1A1 DES F13A1
2 homeostasis/metabolism MP:0005376 10 CD34 COL1A1 DES F13A1 MMP11 PDGFB
3 hematopoietic system MP:0005397 9.91 COL1A1 F13A1 PDGFB PDGFRA PDGFRB TP53
4 muscle MP:0005369 9.61 ACTC1 CD163 COL1A1 DES PDGFB PDGFRA
5 respiratory system MP:0005388 9.17 CD163 COL1A1 F13A1 PDGFB PDGFRA TP53

Drugs & Therapeutics for Dermatofibrosarcoma Protuberans

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

(show all 27)
# 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, Investigational 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 Isophosphamide mustard Phase 3 0
9 Topoisomerase Inhibitors Phase 3,Phase 1
10
Sunitinib Approved, Investigational Phase 2 341031-54-7, 557795-19-4 5329102
11
Paclitaxel Approved, Vet_approved Phase 1, Phase 2 33069-62-4 36314
12
Saracatinib Investigational Phase 2 379231-04-6
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 Albumin-Bound Paclitaxel Phase 1, Phase 2
19 Antimitotic Agents Phase 1, Phase 2
20 Antineoplastic Agents, Phytogenic Phase 1, Phase 2
21 taxane Phase 1, Phase 2
22 tyrosine Nutraceutical Phase 1, Phase 2
23
Cyclophosphamide Approved, Investigational Phase 1 50-18-0, 6055-19-2 2907
24 Antibodies Phase 1
25 Antibodies, Monoclonal Phase 1
26 Immunoglobulins Phase 1
27 Antirheumatic Agents Phase 1

Interventional clinical trials:

(show all 21)

# Name Status NCT ID Phase Drugs
1 Ifosfamide and Doxorubicin, Radiation Therapy, and/or Surgery in Treating Young Patients With Localized Soft Tissue Sarcoma Unknown status NCT00334854 Phase 3 doxorubicin hydrochloride;ifosfamide
2 Observation, Radiation Therapy, Combination Chemotherapy, and/or Surgery in Treating Young Patients With Soft Tissue Sarcoma Completed NCT00346164 Phase 3 doxorubicin hydrochloride;ifosfamide
3 Neoadjuvant Imatinib in Dermatofibrosarcoma Protuberans Completed NCT00243191 Phase 2 imatinib mesylate
4 Imatinib in Dermatofibrosarcoma Protuberans (DFSP) Completed NCT00122473 Phase 1, Phase 2 Imatinib (Glivec)
5 Imatinib Mesylate in Treating Patients With Locally Recurrent or Metastatic Dermatofibrosarcoma Protuberans Completed NCT00084630 Phase 2 imatinib mesylate
6 Imatinib Mesylate in Treating Patients With Locally Advanced or Metastatic Dermatofibrosarcoma Protuberans or Giant Cell Fibroblastoma Completed NCT00085475 Phase 2 imatinib mesylate
7 Imatinib Mesylate (Gleevec) in the Treatment of Systemic Sclerosis Completed NCT00555581 Phase 2 Imatinib Mesylate
8 Imatinib Mesylate in Patients With Various Types of Malignancies Involving Activated Tyrosine Kinase Enzymes Completed NCT00171912 Phase 2 imatinib mesylate
9 Sunitinib in Treating Patients With Metastatic, Locally Advanced, or Locally Recurrent Sarcomas Completed NCT00474994 Phase 2 sunitinib malate
10 Vismodegib and Gamma-Secretase/Notch Signalling Pathway Inhibitor RO4929097 in Treating Patients With Advanced or Metastatic Sarcoma Completed NCT01154452 Phase 1, Phase 2 Gamma-Secretase Inhibitor RO4929097;Vismodegib
11 AZD0530 in Treating Patients With Recurrent Locally Advanced or Metastatic Soft Tissue Sarcoma Completed NCT00659360 Phase 2 saracatinib
12 Phase II Study of Imatinib Mesylate in Patients With Life Threatening Malignant Rare Diseases Completed NCT00154388 Phase 2 Imatinib mesylate
13 To Find a Safe Dose and Show Early Clinical Activity of Weekly Nab-paclitaxel in Pediatric Patients With Recurrent/ Refractory Solid Tumors Active, not recruiting NCT01962103 Phase 1, Phase 2 nab-paclitaxel
14 Phase II Pazopanib Study in Advanced Dermatofibrosarcomas Terminated NCT01059656 Phase 2 Pazopanib
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 Doxorubicin Hydrochloride
17 Imatinib Mesylate And Cyclophosphamide In Metronomic Administration: Dose Escalation Study Of Imatinib Mesylate Completed NCT01046487 Phase 1 Imatinib mesylate, Cyclophosphamide (Dosing level 1 );Imatinib mesylate, Cyclophosphamide (Dosing level 2);Imatinib mesylate, Cyclophosphamide (Dosing level 3)
18 Clinical Outcome of Pediatric Dermatofibrosarcoma Protuberans Unknown status NCT00173355
19 Treatment of Dermatofibrosarcoma Protuberans in Patients 10 Years and Younger Recruiting NCT03381846
20 Spanish Registry of Mohs Surgery Recruiting NCT02310503
21 Glutamine in Preventing Oral Mucositis in Patients Receiving Chemotherapy for Sarcoma Withdrawn NCT00334984 glutamine

Search NIH Clinical Center for Dermatofibrosarcoma Protuberans

Inferred drug relations via UMLS 69 / NDF-RT 47 :


Cochrane evidence based reviews: dermatofibrosarcoma

Genetic Tests for Dermatofibrosarcoma Protuberans

Genetic tests related to Dermatofibrosarcoma Protuberans:

# Genetic test Affiliating Genes
1 Dermatofibrosarcoma Protuberans 28 PDGFB

Anatomical Context for Dermatofibrosarcoma Protuberans

MalaCards organs/tissues related to Dermatofibrosarcoma Protuberans:

38
Skin, Lung, Bone, Lymph Node, Breast, 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 678)
# Title Authors Year
1
Two childhood cases of pigmented dermatofibrosarcoma protuberans with atrophic change. ( 29336320 )
2018
2
Reply to comment on: Orbital dermatofibrosarcoma protuberans with frontal and ethmoid sinus involvement: A case report and brief review of literature. ( 29380814 )
2018
3
Comment on: Orbital dermatofibrosarcoma protuberans with frontal and ethmoid sinus involvement: A case report and brief review of literature. ( 29380813 )
2018
4
Lung Metastasis From Fibrosarcomatous Dermatofibrosarcoma Protuberans of the Vulva: A Rare Case Report. ( 29271862 )
2018
5
A Rare Case of Misdiagnosis: Recurrence of Dermatofibrosarcoma Protuberans That Was Treated Surgicallyas a Keloid. ( 29416224 )
2018
6
Dermatofibrosarcoma Protuberans of Distal Extremities and Acral Sites: A Clinicopathologic Analysis of 27 Cases. ( 29240584 )
2018
7
Dermatofibrosarcoma protuberans in pediatric patients: A diagnostic and management challenge. ( 29387771 )
2018
8
Wide local excision vs. Mohs TA1bingen technique in the treatment of dermatofibrosarcoma protuberans: a two-centre retrospective study and literature review. ( 28573714 )
2017
9
Incidence and Outcomes of Dermatofibrosarcoma Protuberans in the US Pediatric Population. ( 27922973 )
2017
10
Orbitofacial dermatofibrosarcoma protuberans with intranasal extension. ( 29058523 )
2017
11
Operative management of dermatofibrosarcoma protuberans of the breast. ( 28154469 )
2017
12
The Role of CD34 and D2-40 in the Differentiation of Dermatofibroma and Dermatofibrosarcoma Protuberans. ( 28832078 )
2017
13
Sclerosing Dermatofibrosarcoma Protuberans Shows Significant Overlap With Sclerotic Fibroma in Both Routine and Immunohistochemical Analysis: A Potential Diagnostic Pitfall. ( 28134725 )
2017
14
Sequential development of dermatofibrosarcoma protuberans in the forehead and eyelid. ( 29217050 )
2017
15
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
16
Acral dermatofibrosarcoma protuberans with myoid differentiation: A report of 2 cases. ( 28627712 )
2017
17
Dermatofibrosarcoma protuberans of the breast: A case report. ( 28693264 )
2017
18
Unforeseen clonal evolution of tumor cell population in recurrent and metastatic dermatofibrosarcoma protuberans. ( 28977029 )
2017
19
Toluidine Blue Stain of Dermatofibrosarcoma Protuberans: Highlighting Its Use in Mohs. ( 28346254 )
2017
20
Dermatofibrosarcoma protuberans coexisting in a patient with a vascular malformation-a rare coincidence. ( 29026517 )
2017
21
Congenital dermatofibrosarcoma protuberans clinically mimicking a melanocytic naevus treated with serial excisions. ( 28622423 )
2017
22
PDGFB Rearrangements in Dermatofibrosarcoma Protuberans of the Vulva: A Study of 11 Cases Including Myxoid and Fibrosarcomatous Variants. ( 29140881 )
2017
23
Application of COL1A1-PDGFB fusion gene detection by fluorescence inA situ hybridization in biopsy tissue of dermatofibrosarcoma protuberans. ( 28150334 )
2017
24
Long-term results of treatment of advanced dermatofibrosarcoma protuberans (DFSP) with imatinib mesylate - The impact of fibrosarcomatous transformation. ( 28365129 )
2017
25
Dermatofibrosarcoma Protuberans: An Immunomarker Study of 57 Cases That Included Putative Mesenchymal Stem Cell Markers. ( 28362700 )
2017
26
Subcutaneous dermatofibrosarcoma protuberans, a rare subtype with predilection for the head: A retrospective series of 18 cases. ( 28420485 )
2017
27
Orbital dermatofibrosarcoma protuberans with frontal and ethmoid sinus involvement: A case report and brief review of literature. ( 28905843 )
2017
28
Dermatofibrosarcoma protuberans of the scalp: therapeutic challenges. ( 28513108 )
2017
29
The Immunological Roles of Periostin/Tumor-Associated Macrophage Axis in Development of Dermatofibrosarcoma Protuberans. ( 28551623 )
2017
30
When Immunohistochemistry Deceives Us: The Pitfalls of CD34 and Factor XIIIa Stains in Dermatofibroma and Dermatofibrosarcoma Protuberans. ( 28270311 )
2017
31
Association of patient demographic characteristics with Dermatofibrosarcoma Protuberans tumor size at diagnosis in the National Cancer Data Base. ( 28144941 )
2017
32
Dermoscopy of uncommon variants of dermatofibrosarcoma protuberans. ( 28191686 )
2017
33
Phosphohistone-H3 and Ki67: Useful Markers in Differentiating Dermatofibroma From Dermatofibrosarcoma Protuberans and Atypical Fibrohistiocytic Lesions. ( 28609344 )
2017
34
Dermatofibrosarcoma protuberans and gastrointestinal stromal tumor as models for targeted therapy in soft tissue sarcomas. ( 28988501 )
2017
35
Dermatofibrosarcoma Protuberans. ( 29204401 )
2017
36
Recurrence case of rare scalp dermatofibrosarcoma protuberans: Two case reports of a wide radical excision, craniectomy bone involvement followed by cranioplasty and reconstruction. ( 28607816 )
2017
37
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
38
Clinical Features and Treatment of Dermatofibrosarcoma Protuberans Affecting the Vulva: A Literature Review. ( 28323651 )
2017
39
Paediatric dermatofibrosarcoma protuberans (DFSP): Evaluation of a rare childhood malignancy, the Welsh experience. ( 28802546 )
2017
40
Massively Metastatic Dermatofibrosarcoma Protuberans With Fibrosarcomatous Transformation Years After Surgical Resection. ( 28595245 )
2017
41
Dermatofibrosarcoma Protuberans in a 9-Year-Old Child. ( 28584757 )
2017
42
Dermatofibrosarcoma Protuberans. ( 28795284 )
2017
43
Dermatofibrosarcoma Protuberans Presenting as a Large Atrophic Plaque on the Chest. ( 28929166 )
2017
44
Dermatofibrosarcoma protuberans. ( 28873112 )
2017
45
Diagnosis and Management of Dermatofibrosarcoma Protuberans in aA 3-Year-Old Patient. ( 28549894 )
2017
46
Recurrent dermatofibrosarcoma protuberans treated with neoadjuvant imatinib mesylate followed by Mohs micrographic surgery. ( 28971134 )
2017
47
Breast and pleuropulmonary metastasis of multirecurrent scalp dermatofibrosarcoma protuberans: a case report. ( 28388964 )
2017
48
Programmed death-ligand 1 expression is associated with fibrosarcomatous transformation of dermatofibrosarcoma protuberans. ( 28515919 )
2017
49
Clinical Features, Pathological Findings and Treatment of Recurrent Dermatofibrosarcoma Protuberans. ( 28607608 )
2017
50
Survival in patients with primary Dermatofibrosarcoma Protuberans: National Cancer Data Base analysis. ( 29175214 )
2017

Variations for Dermatofibrosarcoma Protuberans

ClinVar genetic disease variations for Dermatofibrosarcoma Protuberans:

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

Cosmic variations for Dermatofibrosarcoma Protuberans:

9
# 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 18)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.77 COL1A1 PDGFB PDGFRA PDGFRB TP53
2
Show member pathways
12.47 PDGFB PDGFRA PDGFRB TP53
3
Show member pathways
12.4 PDGFB PDGFRA PDGFRB TP53
4 12.24 PDGFB PDGFRA PDGFRB TP53 VIM
5 12.2 PDGFB PDGFRA PDGFRB TP53
6 11.83 NES PDGFB PDGFRB VIM
7 11.79 F13A1 TP53 VIM
8
Show member pathways
11.75 DES NES VIM
9 11.68 CD34 DES PDGFB
10 11.63 PDGFB PDGFRA PDGFRB
11 11.6 PDGFB PDGFRA PDGFRB TP53
12 11.51 ACTC1 DES VIM
13 11.45 PDGFRA PDGFRB TP53
14 11.25 NES PDGFB PDGFRA S100B VIM
15 11.23 PDGFB PDGFRA PDGFRB
16 10.7 COL1A1 DES PDGFB PDGFRA PDGFRB VIM
17 10.69 PDGFB PDGFRA PDGFRB
18 10.07 COL1A1 PDGFB PDGFRA PDGFRB

GO Terms for Dermatofibrosarcoma Protuberans

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

# Name GO ID Score Top Affiliating Genes
1 extracellular region GO:0005576 9.61 APOD CD163 CD34 COL1A1 F13A1 MMP11
2 platelet alpha granule lumen GO:0031093 9.33 F13A1 PDGFB SERPINA3
3 extracellular matrix GO:0031012 9.02 COL1A1 MMP11 NES PDGFB VIM
4 cytoplasm GO:0005737 10.03 ACTC1 CD34 COL1A1 DES F13A1 NES

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

(show all 29)
# Name GO ID Score Top Affiliating Genes
1 response to drug GO:0042493 9.92 ACTC1 APOD COL1A1 TP53
2 positive regulation of gene expression GO:0010628 9.91 ACTC1 CD34 PDGFB TP53 VIM
3 positive regulation of cell migration GO:0030335 9.85 COL1A1 PDGFB PDGFRA PDGFRB
4 platelet degranulation GO:0002576 9.82 F13A1 PDGFB SERPINA3
5 phosphatidylinositol phosphorylation GO:0046854 9.79 PDGFB PDGFRA PDGFRB
6 cell chemotaxis GO:0060326 9.74 PDGFB PDGFRA PDGFRB
7 positive regulation of phosphatidylinositol 3-kinase signaling GO:0014068 9.73 PDGFB PDGFRA PDGFRB
8 positive regulation of fibroblast proliferation GO:0048146 9.7 PDGFB PDGFRA PDGFRB
9 muscle filament sliding GO:0030049 9.65 ACTC1 DES VIM
10 intermediate filament organization GO:0045109 9.63 DES VIM
11 positive regulation of calcium ion import GO:0090280 9.63 PDGFB PDGFRB
12 positive regulation of DNA biosynthetic process GO:2000573 9.61 PDGFB PDGFRB
13 positive regulation of chemotaxis GO:0050921 9.61 PDGFB PDGFRB
14 stem cell proliferation GO:0072089 9.6 CD34 NES
15 retina vasculature development in camera-type eye GO:0061298 9.59 PDGFRA PDGFRB
16 positive regulation of phospholipase C activity GO:0010863 9.58 PDGFRA PDGFRB
17 negative regulation of platelet activation GO:0010544 9.58 PDGFB PDGFRA
18 platelet-derived growth factor receptor signaling pathway GO:0048008 9.58 PDGFB PDGFRA PDGFRB
19 wound healing GO:0042060 9.56 COL1A1 PDGFRA PDGFRB TP53
20 paracrine signaling GO:0038001 9.54 CD34 PDGFB
21 positive regulation of phosphatidylinositol 3-kinase activity GO:0043552 9.54 PDGFB PDGFRA PDGFRB
22 positive regulation of reactive oxygen species metabolic process GO:2000379 9.5 PDGFB PDGFRB TP53
23 metanephric glomerular capillary formation GO:0072277 9.49 PDGFRA PDGFRB
24 positive regulation of metanephric mesenchymal cell migration by platelet-derived growth factor receptor-beta signaling pathway GO:0035793 9.46 PDGFB PDGFRB
25 positive regulation of cell proliferation by VEGF-activated platelet derived growth factor receptor signaling pathway GO:0038091 9.43 PDGFRA PDGFRB
26 response to hyperoxia GO:0055093 9.33 COL1A1 PDGFRB TP53
27 cardiac myofibril assembly GO:0055003 9.13 ACTC1 PDGFRA PDGFRB
28 negative regulation of platelet-derived growth factor receptor-beta signaling pathway GO:2000587 8.8 PDGFB PDGFRA PDGFRB
29 multicellular organism development GO:0007275 10.11 MMP11 NES PDGFB PDGFRA PDGFRB TP53

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

# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 9.8 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 9.43 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
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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