{"Name":"Desmoplastic infantile ganglioglioma","DiseaseID__c":"GARD:0008648","id":8648,"encodedName":"desmoplastic-infantile-ganglioglioma","IsDeleted":false,"Disease_Name_Full__c":"Desmoplastic infantile ganglioglioma","Xref_IDs__c":"C1321878; C4738; DOID:0081259; MEDGEN:231168; MONDO:0022965","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:0022965","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 of neoplastic astrocytes together with a variable neuronal component. It involves the superficial cerebral cortex and leptomeninges, and often attaches to the dura. Although clinically it presents as a large tumor, it generally has a good prognosis following surgical resection. (Adapted from WHO)","GARD_Name__c":"Desmoplastic infantile ganglioglioma","GARD_Synonym__c":"dig","Curated_Disease_Description_Source__c":"GARD:0008648","Curated_Disease_Description__c":"Desmoplastic infantile gangliogliomas are rare brain tumors that are normally located in the frontal or parietal lobes of the brain. They are usually diagnosed before 18 months of age with most infants presenting with a short duration of symptoms. Although seizures are not commonly observed, a bulging fontanelle, rapid head growth, vomiting, and a sunset sign are usually noted.","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:0022965","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":"Desmoplastic infantile gangliogliomas are rare brain tumors that are normally located in the frontal or parietal lobes of the brain. They are usually diagnosed before 18 months of age with most infants presenting with a short duration of symptoms. Although seizures are not commonly observed, a bulging fontanelle, rapid head growth, vomiting, and a sunset sign are usually noted.","Curated_Disease_Description_Source__c":"GARD:0008648","GARD_Synonym__c":"dig","Name":"Desmoplastic infantile ganglioglioma","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://uts.nlm.nih.gov/uts/umls/concept/C1321878","Source__c":"C1321878","Xref__c":"C1321878"},{"URL__c":"https://www.ebi.ac.uk/ols4/ontologies/doid/classes?obo_id=DOID%3A0081259","Source__c":"MONDO:0022965","Xref__c":"DOID:0081259"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=231168","Source__c":"C1321878","Xref__c":"MEDGEN:231168"},{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C4738","Source__c":"C1321878; MONDO:0022965","Xref__c":"C4738"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0022965","Source__c":"GARD:0008648","Xref__c":"MONDO:0022965"}],"tags":{"Disease Category":["Cancer"]},"synonyms":["dig"]}