{"Name":"Desmoplastic infantile astrocytoma","DiseaseID__c":"GARD:0009617","id":9617,"encodedName":"desmoplastic-infantile-astrocytoma","IsDeleted":false,"Disease_Name_Full__c":"Desmoplastic infantile astrocytoma","Xref_IDs__c":"128787000; C0457179; C9476; MEDGEN:141595; MONDO:0022963","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":1,"Disease_Characteristics_Score__c":1,"No_of_Age_at_Onset__c":0,"Description_Source__c":"MONDO:0022963","Disease_Description__c":"A WHO grade I large cystic tumor that occurs almost exclusively in infants, with a prominent desmoplastic stroma having a neuroepithelial population consisting mainly of neoplastic astrocytes. It involves the superficial cerebral cortex and leptomeninges, and often attaches to the dura. Although clinically it presents as large tumor, it generally has a good prognosis following surgical resection. (Adapted from WHO)","GARD_Name__c":"Desmoplastic infantile astrocytoma","GARD_Synonym__c":"desmoplastic astrocytoma of infancy; dia","Curated_Disease_Description_Source__c":"MONDO:0022963","Curated_Disease_Description__c":"A WHO grade I large cystic tumor that occurs almost exclusively in infants, with a prominent desmoplastic stroma having a neuroepithelial population consisting mainly of neoplastic astrocytes. It involves the superficial cerebral cortex and leptomeninges, and often attaches to the dura. Although clinically it presents as large tumor, it generally has a good prognosis following surgical resection. (Adapted from WHO)","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:0022963","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 WHO grade I large cystic tumor that occurs almost exclusively in infants, with a prominent desmoplastic stroma having a neuroepithelial population consisting mainly of neoplastic astrocytes. It involves the superficial cerebral cortex and leptomeninges, and often attaches to the dura. Although clinically it presents as large tumor, it generally has a good prognosis following surgical resection. (Adapted from WHO)","Curated_Disease_Description_Source__c":"MONDO:0022963","GARD_Synonym__c":"desmoplastic astrocytoma of infancy; dia","Name":"Desmoplastic infantile astrocytoma","estimateUsa":""}],"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://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C9476","Source__c":"C0457179; MONDO:0022963","Xref__c":"C9476"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=141595","Source__c":"C0457179","Xref__c":"MEDGEN:141595"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C0457179","Source__c":"C0457179","Xref__c":"C0457179"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=128787000","Source__c":"C0457179","Xref__c":"128787000"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0022963","Source__c":"GARD:0009617","Xref__c":"MONDO:0022963"}],"tags":{"Disease Category":["Cancer"]},"synonyms":["desmoplastic astrocytoma of infancy"," dia"]}