MCID: BDN002
MIFTS: 25

Bednar Tumor

Categories: Rare diseases, Skin diseases

Aliases & Classifications for Bednar Tumor

MalaCards integrated aliases for Bednar Tumor:

Name: Bednar Tumor 20
Pigmented Dermatofibrosarcoma Protuberans 20 70

Classifications:



External Ids:

UMLS 70 C0334464

Summaries for Bednar Tumor

GARD : 20 Bednar tumor is a rare variant of dermatofibrosarcoma protuberans (DFSP), a soft tissue sarcoma 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 ring chromosome and skin sarcoma. 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 brain, skin and appendix, 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 124)
# Related Disease Score Top Affiliating Genes
1 ring chromosome 30.4 PDGFB COL1A1
2 skin sarcoma 30.1 PDGFB COL1A1
3 neurofibroma 30.1 VIM S100B
4 fibrosarcoma 30.0 VIM PDGFB COL1A1
5 hemangioma 29.9 VIM PDGFB ENO2
6 dermatofibrosarcoma protuberans 29.6 VIM S100B PDGFB ENO2 COL1A1
7 neurilemmoma 29.6 VIM S100B ENO2
8 congenital heart defects, hamartomas of tongue, and polysyndactyly 10.3
9 fibroma 10.2
10 lipofibromatosis-like neural tumor 10.2
11 melanotic neurilemmoma 10.2
12 malignant peripheral nerve sheath tumor 10.2
13 tièche-jadassohn nevus 10.1
14 rare tumor 10.1
15 ring chromosome 4 10.1 PDGFB COL1A1
16 infantile myofibromatosis 10.0 VIM PDGFB
17 primitive neuroectodermal tumor of the cervix uteri 10.0 VIM ENO2
18 papillary tumor of the pineal region 10.0 VIM ENO2
19 congenital epulis 10.0 VIM ENO2
20 pseudosarcomatous fibromatosis 10.0 VIM COL1A1
21 epulis 10.0 VIM ENO2
22 ewing sarcoma of bone 10.0 VIM ENO2
23 rhabdoid meningioma 10.0 VIM ENO2
24 clear cell ependymoma 10.0 VIM ENO2
25 spastic paraplegia 3, autosomal dominant 10.0 VIM COL1A1
26 granulosa cell tumor of the ovary 10.0 VIM ENO2
27 ependymoblastoma 10.0 VIM ENO2
28 fanconi anemia, complementation group a 10.0
29 deficiency anemia 10.0
30 connective tissue benign neoplasm 10.0
31 melanoma 10.0
32 soft tissue sarcoma 10.0
33 epithelioid leiomyosarcoma 10.0 VIM ENO2
34 melanotic neuroectodermal tumor 10.0 VIM ENO2
35 hypophosphatasia, childhood 10.0 ENO2 COL1A1
36 astroblastoma 10.0 VIM ENO2
37 mesenchymal chondrosarcoma 10.0 VIM ENO2
38 glomus tumor 10.0 VIM ENO2
39 sertoli cell tumor 9.9 VIM ENO2
40 hydromyelia 9.9 VIM S100B
41 primary hepatic neuroendocrine carcinoma 9.9 VIM S100B
42 skin benign neoplasm 9.9 ENO2 COL1A1
43 angiocentric glioma 9.9 VIM S100B
44 cutaneous leiomyosarcoma 9.9 VIM S100B
45 odontogenic myxoma 9.9 VIM S100B
46 chordoid meningioma 9.9 VIM S100B
47 anaplastic ependymoma 9.9 VIM S100B
48 embryonal sarcoma 9.9 VIM S100B
49 polymorphous low-grade adenocarcinoma 9.9 VIM S100B
50 eccrine porocarcinoma 9.9 VIM S100B

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:

40
Brain, Skin, Appendix, Bone, Heart, Ovary, Cervix

Publications for Bednar Tumor

Articles related to Bednar Tumor:

(show top 50) (show all 75)
# Title Authors PMID Year
1
LMNA-NTRK1 rearranged mesenchymal tumor (lipofibromatosis-like neural tumor) mimicking pigmented dermatofibrosarcoma protuberans. 61
32519338 2021
2
Novel response to neoadjuvant anti-PD1 therapy for a patient with retrocaval melanotic schwannoma. 61
33323721 2021
3
A Bednar tumor in a healthy woman. 61
33088558 2020
4
Pigmented Dermatofibrosarcoma Protuberans: Description of a pediatric case. 61
32730419 2020
5
Pigmented dermatofibrosarcoma protuberans associated with dermal melanocytosis. 61
29611228 2018
6
Dermoscopic features of Bednar tumor: Report of a case. 61
29341231 2018
7
Microphthalmia-associated transcription factor (MiTF): Promiscuous staining patterns in fibrohistiocytic lesions is a potential pitfall. 61
29773427 2018
8
Two childhood cases of pigmented dermatofibrosarcoma protuberans with atrophic change. 61
29336320 2018
9
Subcutaneous dermatofibrosarcoma protuberans, a rare subtype with predilection for the head: A retrospective series of 18 cases. 61
28420485 2017
10
Application of COL1A1-PDGFB fusion gene detection by fluorescence in situ hybridization in biopsy tissue of dermatofibrosarcoma protuberans. 61
28150334 2017
11
Sclerosing Dermatofibrosarcoma Protuberans Shows Significant Overlap With Sclerotic Fibroma in Both Routine and Immunohistochemical Analysis: A Potential Diagnostic Pitfall. 61
28134725 2017
12
Case of pigmented dermatofibrosarcoma protuberans with atrophic change. 61
27029004 2016
13
Bednar Tumor (Pigmented Dermatofibrosarcoma Protuberans). 61
26945477 2016
14
Bednar Tumor Mimicking Congenital Melanocytic Nevus. 61
26875791 2016
15
Non psammomatous melanocytic schwannoma presenting as a subcutaneous nodule: A rare presentation of a rare lesion. 61
27366278 2016
16
Bednar Tumor: An Uncommon Entity. 61
27504443 2016
17
Pigmented Dermatofibrosarcoma Protuberans and Blue Naevi with Similar Dermoscopy: A Case Report. 61
26258458 2016
18
Photoletter to the editor: Pigmented dermatofibrosarcoma protuberans in a 4-year-old girl and ultrasonographic findings. 61
26236415 2015
19
Variant of dermatofibrosarcoma protuberans: bednar tumor. 61
25657441 2015
20
Congenital myxoid and pigmented dermatofibrosarcoma protuberans: a case report. 61
23534369 2013
21
Dermatofibrosarcoma protuberans: a comprehensive review and update on diagnosis and management. 61
23327727 2013
22
[Czech eponyms in pathology]. 61
23432077 2013
23
Congenital Bednar tumor over 10 years. 61
22671303 2012
24
Fibrosarcomatous pigmented dermatofibrosarcoma protuberans: A case report with review of the literature. 61
23741240 2012
25
A case of congenital pigmented dermatofibrosarcoma protuberans (Bednar tumor) in a patient with Fanconi anemia. 61
21438918 2011
26
Skin metastasis of dermatofibrosarcoma protuberans with distinct morphological features, confirmed by COL1A1-PDGFB fusion gene analysis. 61
19539850 2009
27
A firm red-brown plaque on the arm. Dermatofibrosarcoma protuberans(DFSP), pigmented variant (Bednar tumor. 61
19451510 2009
28
CD34+ pigmented fibrous proliferations: the morphologic overlap between pigmented dermatofibromas and Bednar tumors. 61
18806495 2008
29
Pigmented dermatofibrosarcoma protuberans with prominent meningothelial-like whorls. 61
18544057 2008
30
Congenital Bednar tumour (pigmented dermatofibrosarcoma protuberans). 61
18363923 2008
31
Pigmented dermatofibrosarcoma protuberance (Bednar tumor). 61
18086613 2008
32
A clinical, histologic, and molecular study of 9 cases of congenital dermatofibrosarcoma protuberans. 61
17310000 2007
33
[From cytogenetics to cytogenomics of dermatofibrosarcoma protuberans family of tumors]. 61
17337387 2007
34
Polypoid dermal dendrocytic hamartoma in a giant congenital melanocytic nevus. 61
17172504 2007
35
Tumoral, quasitumoral and pseudotumoral lesions of the superficial and somatic soft tissue: new entities and new variants of old entities recorded during the last 25 years. Part XII: appendix. 61
17175794 2006
36
COL1A1-PDGFB fusion in a pediatric Bednar tumor with 2 copies of a der(22)t(17;22). 61
16843106 2006
37
[Melanoma simulating malignant soft tissue tumour]. 61
16382990 2005
38
Bednar tumor. 61
15983708 2005
39
[Diagnostic image (237). A man with a swelling on his left buttock. Bednar tumor]. 61
15903039 2005
40
Bednar tumor. 61
15711690 2004
41
An unusual pigmented skin tumour. Bednar Tumour, dorsum of left foot (pigmented dermatofibrosarcoma protuberans). 61
15370137 2004
42
Bednar tumor: a report of two cases. 61
15235190 2004
43
Fibrosarcomatous Bednar tumor with distant metastases--a case report. 61
15471119 2004
44
Metastatic Bednar tumor (pigmented dermatofibrosarcoma protuberans) with fibrosarcomatous change: a case report. 61
16228691 2004
45
Genetics of dermatofibrosarcoma protuberans family of tumors: from ring chromosomes to tyrosine kinase inhibitor treatment. 61
12661001 2003
46
Bednar tumor: report of a case with immunohistochemical and ultrastructural study. 61
12775510 2003
47
Bednár tumor associated with dermal melanocytosis: melanocytic colonization or neuroectodermal multidirectional differentiation? 61
12641795 2003
48
Pigmented dermatofibrosarcoma protuberans of the foot with fibrosarcomatous changes: a review and case presentation. 61
12075908 2002
49
EMA+ cells in dermatofibrosarcoma protuberans. A study of 11 tumors suggesting perineurial cell differentiation. 61
12426982 2002
50
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. 61
12034531 2002

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 S100B PDGFB ENO2 COL1A1

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 VIM S100B PDGFB COL1A1
2 platelet-derived growth factor binding GO:0048407 8.62 PDGFB COL1A1

Sources for Bednar Tumor

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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