{"Name":"Neurofibrosarcoma","DiseaseID__c":"GARD:0008211","id":8211,"encodedName":"neurofibrosarcoma","IsDeleted":false,"Disease_Name_Full__c":"Neurofibrosarcoma","Xref_IDs__c":"C0206729; D018319; DOID:3512; HP:0100697; MEDGEN:104927; MONDO:0002675","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":1,"No_of_Age_at_Onset__c":0,"Description_Source__c":"MONDO:0002675","Disease_Description__c":"A malignant tumor that arises from small cutaneous nerves, is locally aggressive, and has a potential for metastasis. Characteristic histopathologic features include proliferating atypical spindle cells with slender wavy and pointed nuclei, hypocellular areas, and areas featuring organized whorls of fibroblastic proliferation. The most common primary sites are the extremities, retroperitoneum, and trunk. These tumors tend to present in childhood, often in association with neurofibromatosis 1. (From DeVita et al., Cancer: Principles & Practice of Oncology, 5th ed, p1662; Mayo Clin Proc 1990 Feb;65(2):164-72)","GARD_Name__c":"Neurofibrosarcoma","GARD_Synonym__c":"malignant peripheral nerve sheath tumour; malignant schwannoma; neurosarcoma; neurosarcoma [obs]","Curated_Disease_Description_Source__c":"MONDO:0002675","Curated_Disease_Description__c":"A malignant tumor that arises from small cutaneous nerves, is locally aggressive, and has a potential for metastasis. Characteristic histopathologic features include proliferating atypical spindle cells with slender wavy and pointed nuclei, hypocellular areas, and areas featuring organized whorls of fibroblastic proliferation. The most common primary sites are the extremities, retroperitoneum, and trunk. These tumors tend to present in childhood, often in association with neurofibromatosis 1. (From DeVita et al., Cancer: Principles & Practice of Oncology, 5th ed, p1662; Mayo Clin Proc 1990 Feb;65(2):164-72)","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:0002675","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":"A malignant tumor that arises from small cutaneous nerves, is locally aggressive, and has a potential for metastasis. Characteristic histopathologic features include proliferating atypical spindle cells with slender wavy and pointed nuclei, hypocellular areas, and areas featuring organized whorls of fibroblastic proliferation. The most common primary sites are the extremities, retroperitoneum, and trunk. These tumors tend to present in childhood, often in association with neurofibromatosis 1. (From DeVita et al., Cancer: Principles & Practice of Oncology, 5th ed, p1662; Mayo Clin Proc 1990 Feb;65(2):164-72)","Curated_Disease_Description_Source__c":"MONDO:0002675","GARD_Synonym__c":"malignant peripheral nerve sheath tumour; malignant schwannoma; neurosarcoma; neurosarcoma [obs]","Name":"Neurofibrosarcoma","estimateUsa":""}],"Organization_Supported_Diseases__c":[{"Account_Name__c":"Sarcoma Alliance for Research through Collaboration","Website__c":"https://sarctrials.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://uts.nlm.nih.gov/uts/umls/concept/C0206729","Source__c":"C0206729","Xref__c":"C0206729"},{"URL__c":"https://www.ebi.ac.uk/ols4/ontologies/doid/classes?obo_id=DOID%3A3512","Source__c":"MONDO:0002675","Xref__c":"DOID:3512"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=104927","Source__c":"C0206729","Xref__c":"MEDGEN:104927"},{"URL__c":"https://www.ncbi.nlm.nih.gov/mesh/C018319","Source__c":"C0206729; MONDO:0002675","Xref__c":"D018319"},{"URL__c":"https://hpo.jax.org/browse/term/HP:0100697","Source__c":"C0206729","Xref__c":"HP:0100697"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0002675","Source__c":"GARD:0008211","Xref__c":"MONDO:0002675"}],"tags":{"Disease Category":["Cancer"]},"synonyms":["malignant peripheral nerve sheath tumour"," malignant schwannoma"," neurosarcoma"," neurosarcoma [obs]"]}