MCID: BDN002
MIFTS: 28

Bednar Tumor

Categories: Rare diseases, Skin diseases

Aliases & Classifications for Bednar Tumor

MalaCards integrated aliases for Bednar Tumor:

Name: Bednar Tumor 54
Pigmented Dermatofibrosarcoma Protuberans 54 74

Classifications:



External Ids:

UMLS 74 C0334464

Summaries for Bednar Tumor

NIH Rare Diseases : 54 Bednar tumor is a rare variant of dermatofibrosarcoma protuberans (DFSP), a soft tissuesarcoma that develops in the deep layers of the skin. It accounts for approximately 1% of all DFSP cases. Bednar tumor is also known as pigmented DFSP because it contains dark-colored cells that give may give the tumor a multi-colored (i.e red and brown) appearance. The tumor may begin as a painless, slow-growing papule or patch of skin; however, accelerated growth, bleeding and/or pain are often observed as it grows. The underlying cause of Bednar tumor is unknown. There is currently no evidence of an inherited risk for the condition and most cases occur sporadically in people with no family history of the condition. Treatment varies based on the severity of the condition, the location of the tumor and the overall health of the affected person. The tumor is generally treated with surgery. In advanced cases, radiation therapy and/or systemic therapy may be recommended, as well.

MalaCards based summary : Bednar Tumor, also known as pigmented dermatofibrosarcoma protuberans, is related to dermatofibrosarcoma protuberans and ring chromosome 4. An important gene associated with Bednar Tumor is PDGFB (Platelet Derived Growth Factor Subunit B), and among its related pathways/superpathways are Embryonic and Induced Pluripotent Stem Cell Differentiation Pathways and Lineage-specific Markers and Neural Stem Cell Differentiation Pathways and Lineage-specific Markers. Affiliated tissues include skin, brain and colon, and related phenotype is respiratory system.

Related Diseases for Bednar Tumor

Diseases related to Bednar Tumor via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 96)
# Related Disease Score Top Affiliating Genes
1 dermatofibrosarcoma protuberans 30.1 COL1A1 PDGFB S100B VIM
2 ring chromosome 4 10.2 COL1A1 PDGFB
3 dermis tumor 10.2 COL1A1 PDGFB
4 adult fibrosarcoma 10.2 COL1A1 PDGFB
5 focal hand dystonia 10.1 PDGFB VIM
6 skin sarcoma 10.1 COL1A1 PDGFB
7 fanconi anemia, complementation group a 10.1
8 neurofibroma 10.1
9 primitive neuroectodermal tumor of the cervix uteri 10.1 ENO2 VIM
10 papillary tumor of the pineal region 10.1 ENO2 VIM
11 epulis 10.1 ENO2 VIM
12 epithelioid leiomyosarcoma 10.1 ENO2 VIM
13 clear cell ependymoma 10.1 ENO2 VIM
14 bone ewing's sarcoma 10.1 ENO2 PDGFB
15 astroblastoma 10.1 ENO2 VIM
16 melanotic neuroectodermal tumor 10.1 ENO2 VIM
17 desmoplastic small round cell tumor 10.1 ENO2 VIM
18 extraskeletal ewing sarcoma 10.0 ENO2 VIM
19 glomus tumor 10.0 ENO2 VIM
20 chondroid chordoma 10.0 ENO2 VIM
21 liver angiosarcoma 10.0 ENO2 VIM
22 congenital epulis 10.0 ENO2 VIM
23 granulosa cell tumor of the ovary 10.0 ENO2 VIM
24 pericardial mesothelioma 10.0 ENO2 VIM
25 fibrous histiocytoma 10.0 S100B VIM
26 skin benign neoplasm 10.0 COL1A1 ENO2
27 hydromyelia 10.0 S100B VIM
28 primary hepatic neuroendocrine carcinoma 10.0 S100B VIM
29 syringocystadenoma papilliferum 10.0 S100B VIM
30 malignant peripheral nerve sheath tumor 10.0 S100B VIM
31 fibrous meningioma 10.0 S100B VIM
32 chordoid meningioma 10.0 S100B VIM
33 eccrine porocarcinoma 10.0 S100B VIM
34 myxopapillary ependymoma 9.9 S100B VIM
35 monophasic synovial sarcoma 9.9 S100B VIM
36 syringoma 9.9 S100B VIM
37 angiocentric glioma 9.9 S100B VIM
38 juvenile xanthogranuloma 9.9 S100B VIM
39 pleomorphic liposarcoma 9.9 S100B VIM
40 amelanotic melanoma 9.9 S100B VIM
41 anaplastic ependymoma 9.9 S100B VIM
42 perineurioma 9.9 S100B VIM
43 spindle cell sarcoma 9.9 S100B VIM
44 odontogenic myxoma 9.9 S100B VIM
45 chordoma 9.9 S100B VIM
46 dendritic cell tumor 9.9 S100B VIM
47 embryonal sarcoma 9.9 S100B VIM
48 alveolar soft part sarcoma 9.9 ENO2 VIM
49 intracranial cysts 9.9 ENO2 S100B
50 epithelioid malignant peripheral nerve sheath tumor 9.9 ENO2 S100B

Graphical network of the top 20 diseases related to Bednar Tumor:



Diseases related to Bednar Tumor

Symptoms & Phenotypes for Bednar Tumor

MGI Mouse Phenotypes related to Bednar Tumor:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 respiratory system MP:0005388 8.92 COL1A1 ENO2 PDGFB VIM

Drugs & Therapeutics for Bednar Tumor

Search Clinical Trials , NIH Clinical Center for Bednar Tumor

Genetic Tests for Bednar Tumor

Anatomical Context for Bednar Tumor

MalaCards organs/tissues related to Bednar Tumor:

42
Skin, Brain, Colon, Liver, Bone, Ovary, Cervix

Publications for Bednar Tumor

Articles related to Bednar Tumor:

(show all 50)
# Title Authors Year
1
Dermoscopic features of Bednar tumor: Report of a case. ( 29341231 )
2018
2
Two childhood cases of pigmented dermatofibrosarcoma protuberans with atrophic change. ( 29336320 )
2018
3
Pigmented dermatofibrosarcoma protuberans associated with dermal melanocytosis. ( 29611228 )
2018
4
Bednar Tumor Mimicking Congenital Melanocytic Nevus. ( 26875791 )
2016
5
Bednar Tumor (Pigmented Dermatofibrosarcoma Protuberans). ( 26945477 )
2016
6
Bednar Tumor: An Uncommon Entity. ( 27504443 )
2016
7
Pigmented Dermatofibrosarcoma Protuberans and Blue Naevi with Similar Dermoscopy: A Case Report. ( 26258458 )
2016
8
Case of pigmented dermatofibrosarcoma protuberans with atrophic change. ( 27029004 )
2016
9
Variant of dermatofibrosarcoma protuberans: bednar tumor. ( 25657441 )
2015
10
Photoletter to the editor: Pigmented dermatofibrosarcoma protuberans in a 4-year-old girl and ultrasonographic findings. ( 26236415 )
2015
11
Congenital myxoid and pigmented dermatofibrosarcoma protuberans: a case report. ( 23534369 )
2013
12
Congenital Bednar tumor over 10 years. ( 22671303 )
2012
13
Fibrosarcomatous pigmented dermatofibrosarcoma protuberans: A case report with review of the literature. ( 23741240 )
2012
14
A case of congenital pigmented dermatofibrosarcoma protuberans (Bednar tumor) in a patient with Fanconi anemia. ( 21438918 )
2011
15
A firm red-brown plaque on the arm. Dermatofibrosarcoma protuberans(DFSP), pigmented variant (Bednar tumor. ( 19451510 )
2009
16
Pigmented dermatofibrosarcoma protuberance (Bednar tumor). ( 18086613 )
2008
17
Congenital Bednar tumour (pigmented dermatofibrosarcoma protuberans). ( 18363923 )
2008
18
Pigmented dermatofibrosarcoma protuberans with prominent meningothelial-like whorls. ( 18544057 )
2008
19
COL1A1-PDGFB fusion in a pediatric Bednar tumor with 2 copies of a der(22)t(17;22). ( 16843106 )
2006
20
Bednar tumor. ( 15983708 )
2005
21
Bednar tumor: a report of two cases. ( 15235190 )
2004
22
Fibrosarcomatous Bednar tumor with distant metastases--a case report. ( 15471119 )
2004
23
Bednar tumor. ( 15711690 )
2004
24
Metastatic Bednar tumor (pigmented dermatofibrosarcoma protuberans) with fibrosarcomatous change: a case report. ( 16228691 )
2004
25
An unusual pigmented skin tumour. Bednar Tumour, dorsum of left foot (pigmented dermatofibrosarcoma protuberans). ( 15370137 )
2004
26
Bednár tumor associated with dermal melanocytosis: melanocytic colonization or neuroectodermal multidirectional differentiation? ( 12641795 )
2003
27
Bednar tumor: report of a case with immunohistochemical and ultrastructural study. ( 12775510 )
2003
28
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 )
2002
29
Pediatric pigmented dermatofibrosarcoma protuberans (Bednár tumor): case report and review of the literature with emphasis on the differential diagnosis. ( 12440785 )
2002
30
Composite dermatofibrosarcoma protuberans-giant cell fibroblastoma recurring as Bednár tumor-giant cell fibroblastoma with mucoid lakes and with amputation neuroma. ( 12629865 )
2002
31
Pigmented dermatofibrosarcoma protuberans of the foot with fibrosarcomatous changes: a review and case presentation. ( 12075908 )
2002
32
Supernumerary ring chromosome in a Bednar tumor (pigmented dermatofibrosarcoma protuberans) is composed of interspersed sequences from chromosomes 17 and 22: a fluorescence in situ hybridization and comparative genomic hybridization analysis. ( 11170290 )
2001
33
Bednar tumor of the foot: a case report. ( 11354449 )
2001
34
Bednar tumor (pigmented dermatofibrosarcoma protuberans) occurring in a site of prior immunization: immunochemical findings and therapy. ( 10025857 )
1999
35
An ultrastructural and immunohistochemical study of pigmented dermatofibrosarcoma protuberans (Bednar tumor). ( 10417439 )
1999
36
Pigmented dermatofibrosarcoma protuberans (Bednár tumor) occurring in a Japanese infant. ( 9693186 )
1998
37
Dermatofibrosarcoma protuberans and the Bednar tumor: treatment in the pediatric population. ( 9869058 )
1998
38
A case of pigmented dermatofibrosarcoma protuberans (Bednar tumor). ( 9241967 )
1997
39
A case of Bednar tumor. Immunohistochemical positivity for CD34. ( 9267742 )
1997
40
A case of Bednar tumor recurring after 23 years. ( 8916661 )
1996
41
Pigmented dermatofibrosarcoma protuberans (Bednar tumour). ( 8755130 )
1996
42
Giant-cell fibroblastoma with pigmented dermatofibrosarcoma protuberans component. ( 8017567 )
1994
43
Bednar tumor pattern in recurring giant cell fibroblastoma. ( 8356948 )
1993
44
Pigmented dermatofibrosarcoma protuberans (Bednar tumour). ( 1498394 )
1992
45
Storiform neurofibroma (Bednar tumor). A case report. ( 1845487 )
1991
46
Pigmented dermatofibrosarcoma protuberans (Bednár tumor). An autopsy case with systemic metastasis. ( 2096596 )
1990
47
Bednar tumor (pigmented dermatofibrosarcoma protuberans). An analysis of six cases. ( 2291405 )
1990
48
Pigmented dermatofibrosarcoma protuberans (Bednar tumour): melanocytic colonization or neuroectodermal differentiation? A clinicopathological and immunohistochemical study. ( 2466752 )
1988
49
Pigmented dermatofibrosarcoma protuberans. Report of two cases as a variant of dermatofibrosarcoma protuberans with partial neural differentiation. ( 3551654 )
1987
50
Pigmented dermatofibrosarcoma protuberans (Bednar tumor). A pathologic, ultrastructural, and immunohistochemical study. ( 3901787 )
1985

Variations for Bednar Tumor

Expression for Bednar Tumor

Search GEO for disease gene expression data for Bednar Tumor.

Pathways for Bednar Tumor

GO Terms for Bednar Tumor

Cellular components related to Bednar Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 extracellular space GO:0005615 8.92 COL1A1 ENO2 PDGFB S100B

Molecular functions related to Bednar Tumor according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 identical protein binding GO:0042802 9.26 COL1A1 PDGFB S100B VIM
2 platelet-derived growth factor binding GO:0048407 8.62 COL1A1 PDGFB

Sources for Bednar Tumor

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
Content
Loading form....