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 53
Pigmented Dermatofibrosarcoma Protuberans 53 73

Classifications:



External Ids:

UMLS 73 C0334464

Summaries for Bednar Tumor

NIH Rare Diseases : 53 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 intracranial cysts. 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 liver, 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 97)
# Related Disease Score Top Affiliating Genes
1 dermatofibrosarcoma protuberans 30.3 COL1A1 PDGFB S100B VIM
2 intracranial cysts 10.1 ENO2 S100B
3 epithelioid malignant peripheral nerve sheath tumor 10.1 ENO2 S100B
4 middle cerebral artery infarction 10.1 ENO2 S100B
5 papillary ependymoma 10.1 ENO2 S100B
6 bone ewing's sarcoma 10.1 ENO2 PDGFB
7 ring chromosome 4 10.1 COL1A1 PDGFB
8 head injury 10.1 ENO2 S100B
9 pineocytoma 10.1 ENO2 S100B
10 dermis tumor 10.1 COL1A1 PDGFB
11 adult fibrosarcoma 10.1 COL1A1 PDGFB
12 spondylosis 10.1 ENO2 S100B
13 subependymal giant cell astrocytoma 10.1 ENO2 S100B
14 meningeal melanocytoma 10.1 ENO2 S100B
15 traumatic brain injury 10.1 ENO2 S100B
16 brain injury 10.1 ENO2 S100B
17 fanconi anemia, complementation group a 10.0
18 neurofibroma 10.0
19 olfactory neuroblastoma 10.0 ENO2 S100B
20 skin sarcoma 10.0 COL1A1 PDGFB
21 acute mountain sickness 10.0 ENO2 S100B
22 extraosseous ewing's sarcoma 10.0 ENO2 S100B
23 pilomatrixoma 10.0 ENO2 S100B
24 ganglioglioma 10.0 ENO2 S100B
25 clear cell sarcoma 10.0 ENO2 S100B
26 secondary progressive multiple sclerosis 10.0 ENO2 S100B
27 neuroma 10.0 ENO2 S100B
28 hemangioblastoma 10.0 ENO2 S100B
29 fibrous histiocytoma 10.0 S100B VIM
30 focal hand dystonia 10.0 PDGFB VIM
31 skin benign neoplasm 10.0 COL1A1 ENO2
32 hydromyelia 10.0 S100B VIM
33 primitive neuroectodermal tumor of the cervix uteri 10.0 ENO2 VIM
34 papillary tumor of the pineal region 10.0 ENO2 VIM
35 primary hepatic neuroendocrine carcinoma 10.0 S100B VIM
36 epulis 10.0 ENO2 VIM
37 syringocystadenoma papilliferum 10.0 S100B VIM
38 epithelioid leiomyosarcoma 10.0 ENO2 VIM
39 malignant peripheral nerve sheath tumor 10.0 S100B VIM
40 fibrous meningioma 10.0 S100B VIM
41 chordoid meningioma 10.0 S100B VIM
42 clear cell ependymoma 10.0 ENO2 VIM
43 eccrine porocarcinoma 10.0 S100B VIM
44 myxopapillary ependymoma 10.0 S100B VIM
45 monophasic synovial sarcoma 10.0 S100B VIM
46 astroblastoma 10.0 ENO2 VIM
47 spiradenoma 10.0 S100B VIM
48 melanotic neuroectodermal tumor 10.0 ENO2 VIM
49 syringoma 10.0 S100B VIM
50 angiocentric glioma 10.0 S100B VIM

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:

46
# 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:

41
Skin, Brain, Liver, Bone, Ovary, Cervix, Pineal

Publications for Bednar Tumor

Articles related to Bednar Tumor:

(show all 48)
# 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: An Uncommon Entity. ( 27504443 )
2016
5
Bednar Tumor (Pigmented Dermatofibrosarcoma Protuberans). ( 26945477 )
2016
6
Case of pigmented dermatofibrosarcoma protuberans with atrophic change. ( 27029004 )
2016
7
Bednar Tumor Mimicking Congenital Melanocytic Nevus. ( 26875791 )
2016
8
Pigmented Dermatofibrosarcoma Protuberans and Blue Naevi with Similar Dermoscopy: A Case Report. ( 26258458 )
2015
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
Fibrosarcomatous pigmented dermatofibrosarcoma protuberans: A case report with review of the literature. ( 23741240 )
2012
13
Congenital Bednar tumor over 10 years. ( 22671303 )
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
Congenital Bednar tumour (pigmented dermatofibrosarcoma protuberans). ( 18363923 )
2008
17
Pigmented dermatofibrosarcoma protuberance (Bednar tumor). ( 18086613 )
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
Fibrosarcomatous Bednar tumor with distant metastases--a case report. ( 15471119 )
2004
22
Bednar tumor: a report of two cases. ( 15235190 )
2004
23
An unusual pigmented skin tumour. Bednar Tumour, dorsum of left foot (pigmented dermatofibrosarcoma protuberans). ( 15370137 )
2004
24
Bednar tumor. ( 15711690 )
2004
25
Metastatic Bednar tumor (pigmented dermatofibrosarcoma protuberans) with fibrosarcomatous change: a case report. ( 16228691 )
2004
26
Bednar tumor: report of a case with immunohistochemical and ultrastructural study. ( 12775510 )
2003
27
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
28
Pediatric pigmented dermatofibrosarcoma protuberans (BednA!r tumor): case report and review of the literature with emphasis on the differential diagnosis. ( 12440785 )
2002
29
Pigmented dermatofibrosarcoma protuberans of the foot with fibrosarcomatous changes: a review and case presentation. ( 12075908 )
2002
30
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
31
Bednar tumor of the foot: a case report. ( 11354449 )
2001
32
An ultrastructural and immunohistochemical study of pigmented dermatofibrosarcoma protuberans (Bednar tumor). ( 10417439 )
1999
33
Bednar tumor (pigmented dermatofibrosarcoma protuberans) occurring in a site of prior immunization: immunochemical findings and therapy. ( 10025857 )
1999
34
Dermatofibrosarcoma protuberans and the Bednar tumor: treatment in the pediatric population. ( 9869058 )
1998
35
Pigmented dermatofibrosarcoma protuberans (BednA!r tumor) occurring in a Japanese infant. ( 9693186 )
1998
36
A case of pigmented dermatofibrosarcoma protuberans (Bednar tumor). ( 9241967 )
1997
37
A case of Bednar tumor. Immunohistochemical positivity for CD34. ( 9267742 )
1997
38
A case of Bednar tumor recurring after 23 years. ( 8916661 )
1996
39
Pigmented dermatofibrosarcoma protuberans (Bednar tumour). ( 8755130 )
1996
40
Giant-cell fibroblastoma with pigmented dermatofibrosarcoma protuberans component. ( 8017567 )
1994
41
Bednar tumor pattern in recurring giant cell fibroblastoma. ( 8356948 )
1993
42
Pigmented dermatofibrosarcoma protuberans (Bednar tumour). ( 1498394 )
1992
43
Storiform neurofibroma (Bednar tumor). A case report. ( 1845487 )
1991
44
Pigmented dermatofibrosarcoma protuberans (Bednár tumor). An autopsy case with systemic metastasis. ( 2096596 )
1990
45
Bednar tumor (pigmented dermatofibrosarcoma protuberans). An analysis of six cases. ( 2291405 )
1990
46
Pigmented dermatofibrosarcoma protuberans (Bednar tumour): melanocytic colonization or neuroectodermal differentiation? A clinicopathological and immunohistochemical study. ( 2466752 )
1988
47
Pigmented dermatofibrosarcoma protuberans. Report of two cases as a variant of dermatofibrosarcoma protuberans with partial neural differentiation. ( 3551654 )
1987
48
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

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74 UMLS via Orphanet
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