{"Name":"Pigmented dermatofibrosarcoma protuberans","DiseaseID__c":"GARD:0009624","id":9624,"encodedName":"pigmented-dermatofibrosarcoma-protuberans","IsDeleted":false,"Disease_Name_Full__c":"Pigmented dermatofibrosarcoma protuberans","Xref_IDs__c":"398670003; 62621002; C0334464; C9430; MEDGEN:90786; MONDO:0023273","USA_Estimate__c":null,"No_of_Specialist_Tagsa__c":0,"No_of_ClinGen_records__c":0,"No_of_GeneReviews__c":0,"No_of_HHS_records__c":0,"World_Estimate__c":null,"No_of_HRSA_records__c":0,"Evidence_Based_Score__c":0,"No_of_Disease_Descriptions__c":2,"Disease_Characteristics_Score__c":2,"No_of_Age_at_Onset__c":0,"Description_Source__c":"MONDO:0023273","Disease_Description__c":"A morphologic variant of dermatofibrosarcoma protuberans characterized by the presence of melanin-pigmented dendritic cells.","GARD_Name__c":"Pigmented dermatofibrosarcoma protuberans","GARD_Synonym__c":"bednar tumor; bednar tumour; pigmented dermatofibrosarcoma; pigmented dermatofibrosarcoma protuberans (bednar tumor); pigmented dermatofibrosarcoma protuberans of skin; pigmented storiform neurofibroma","Curated_Disease_Description_Source__c":"GARD:0009624","Curated_Disease_Description__c":"Bednar tumor is a rare variant of dermatofibrosarcoma protuberans (DFSP), a soft tissue sarcoma that develops in the deep layers of the skin. 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 may be 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 in people who have no family history of the condition.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":null,"Age_at_Onset_Snippet_Text__c":null,"SourceID__c":null,"Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Entity","MONDO_ID__c":"MONDO:0023273","ORPHANET_ID__c":null,"Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":null,"Spanish_Description_Source__c":null,"Spanish_Description__c":null,"Spanish_Disease_Name__c":null,"Spanish_GARD_Synonym__c":null,"Category_Linearization__c":null,"icd10_id__c":null,"mesh_id__c":null,"omim_id__c":null,"snomed_id__c":null,"umls_id__c":null,"GARD_Disease__c":[{"Curated_Disease_Description__c":"Bednar tumor is a rare variant of dermatofibrosarcoma protuberans (DFSP), a soft tissue sarcoma that develops in the deep layers of the skin. 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 may be 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 in people who have no family history of the condition.","Curated_Disease_Description_Source__c":"GARD:0009624","GARD_Synonym__c":"bednar tumor; bednar tumour; pigmented dermatofibrosarcoma; pigmented dermatofibrosarcoma protuberans (bednar tumor); pigmented dermatofibrosarcoma protuberans of skin; pigmented storiform neurofibroma","Name":"Pigmented dermatofibrosarcoma protuberans","estimateUsa":""}],"Organization_Supported_Diseases__c":[{"Account_Name__c":"Sarcoma Foundation of America","Website__c":"https://www.curesarcoma.org/"},{"Account_Name__c":"Sarcoma Alliance","Website__c":"https://sarcomaalliance.org/"}],"GARD_Disease_Tag__c":[{"Tag_Name__c":"Cancer","Tag_Category__c":"Disease Category","category_description":"Cancer is a disease in which some of the body's cells grow uncontrollably and can spread to other parts of the body.","curated_tag_name":"Cancer"}],"External_Identifier_Disease__c":[{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=90786","Source__c":"C0334464","Xref__c":"MEDGEN:90786"},{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C9430","Source__c":"C0334464; MONDO:0023273","Xref__c":"C9430"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=398670003","Source__c":"C0334464; MONDO:0023273","Xref__c":"398670003"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C0334464","Source__c":"C0334464","Xref__c":"C0334464"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=62621002","Source__c":"C0334464","Xref__c":"62621002"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0023273","Source__c":"GARD:0009624","Xref__c":"MONDO:0023273"}],"tags":{"Disease Category":["Cancer"]},"synonyms":["bednar tumor"," bednar tumour"," pigmented dermatofibrosarcoma"," pigmented dermatofibrosarcoma protuberans (bednar tumor)"," pigmented dermatofibrosarcoma protuberans of skin"," pigmented storiform neurofibroma"]}