{"Name":"Pleomorphic xanthoastrocytoma","DiseaseID__c":"GARD:0010631","id":10631,"encodedName":"pleomorphic-xanthoastrocytoma","IsDeleted":false,"Disease_Name_Full__c":"Pleomorphic xanthoastrocytoma","Xref_IDs__c":"189924002; 78838008; C0334586; C4323; DOID:4852; HP:0033682; MEDGEN:137786; MONDO:0016690; ORPHA:251607","USA_Estimate__c":"1,000","No_of_Specialist_Tagsa__c":3,"No_of_ClinGen_records__c":0,"No_of_GeneReviews__c":0,"No_of_HHS_records__c":0,"World_Estimate__c":"1 to 8,000","No_of_HRSA_records__c":0,"Evidence_Based_Score__c":0,"No_of_Disease_Descriptions__c":3,"Disease_Characteristics_Score__c":4,"No_of_Age_at_Onset__c":1,"Description_Source__c":"MONDO:0016690","Disease_Description__c":"A WHO grade ll astrocytic tumor with a relatively favorable prognosis. It is characterized by pleomorphic and lipidized cells expressing GFAP often surrounded by a reticulin network and eosinophilic granular bodies. It presents in the superficial cerebral hemispheres and involves the meninges. It typically affects children and young adults.","GARD_Name__c":"Pleomorphic xanthoastrocytoma","GARD_Synonym__c":"pleomorphic xantho-astrocytoma; pxa","Curated_Disease_Description_Source__c":"MONDO:0016690","Curated_Disease_Description__c":"A WHO grade ll astrocytic tumor with a relatively favorable prognosis. It is characterized by pleomorphic and lipidized cells expressing GFAP often surrounded by a reticulin network and eosinophilic granular bodies. It presents in the superficial cerebral hemispheres and involves the meninges. It typically affects children and young adults.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":"1,000","Age_at_Onset_Snippet_Text__c":"at any time in life","SourceID__c":"ORPHA:251607","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Entity","MONDO_ID__c":"MONDO:0016690","ORPHANET_ID__c":"ORPHA:251607","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Xantoastrocitoma pleomórfico","Spanish_Description_Source__c":null,"Spanish_Description__c":null,"Spanish_Disease_Name__c":"xantoastrocitoma pleomórfico","Spanish_GARD_Synonym__c":"pxa","Category_Linearization__c":"ORPHA:250908","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 ll astrocytic tumor with a relatively favorable prognosis. It is characterized by pleomorphic and lipidized cells expressing GFAP often surrounded by a reticulin network and eosinophilic granular bodies. It presents in the superficial cerebral hemispheres and involves the meninges. It typically affects children and young adults.","Curated_Disease_Description_Source__c":"MONDO:0016690","GARD_Synonym__c":"pleomorphic xantho-astrocytoma; pxa","Name":"Pleomorphic xanthoastrocytoma","Curated_USA_Estimate__c":"1,000","estimateUsa":"1,000"}],"Organization_Supported_Diseases__c":[{"Account_Name__c":"A Kids’ Brain Tumor Cure Foundation","Website__c":"https://akidsbraintumorcure.org/"},{"Account_Name__c":"Pediatric Brain Tumor Foundation","Website__c":"https://www.curethekids.org/"}],"GARD_Disease_Tag__c":[{"Tag_Name__c":"Cancer - Oncologist","Tag_Category__c":"Specialist"},{"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"},{"Tag_Name__c":"Neurology","Tag_Category__c":"Disease Category;Specialist","category_description":"Neurological diseases affect the brain, spinal cord, cranial nerves, autonomic nerves, or other peripheral nerves.","curated_tag_name":"Neurological diseases"},{"Tag_Name__c":"Pediatrics","Tag_Category__c":"Specialist"}],"Age_At_Onset__c":[{"Age_At_Onset__c":"All ages","Provided_By__c":"ORPHA:251607"}],"External_Identifier_Disease__c":[{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C0334586","Source__c":"C0334586","Xref__c":"C0334586"},{"URL__c":"https://www.orpha.net/en/disease/detail/251607","Source__c":"C0334586; MONDO:0016690; ORPHA:251607","Xref__c":"ORPHA:251607"},{"URL__c":"https://www.ebi.ac.uk/ols4/ontologies/doid/classes?obo_id=DOID%3A4852","Source__c":"MONDO:0016690","Xref__c":"DOID:4852"},{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C4323","Source__c":"C0334586; MONDO:0016690","Xref__c":"C4323"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=137786","Source__c":"C0334586","Xref__c":"MEDGEN:137786"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=78838008","Source__c":"C0334586","Xref__c":"78838008"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0016690","Source__c":"GARD:0010631","Xref__c":"MONDO:0016690"},{"URL__c":"https://hpo.jax.org/browse/term/HP:0033682","Source__c":"C0334586","Xref__c":"HP:0033682"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=189924002","Source__c":"C0334586","Xref__c":"189924002"}],"tags":{"Specialist":["Cancer - Oncologist","Neurology","Pediatrics"],"Disease Category":["Cancer","Neurology"]},"synonyms":["pleomorphic xantho-astrocytoma"," pxa"]}