{"Name":"Inflammatory myofibroblastic tumor","DiseaseID__c":"GARD:0007146","id":7146,"encodedName":"inflammatory-myofibroblastic-tumor","IsDeleted":false,"Disease_Name_Full__c":"Inflammatory myofibroblastic tumor","Xref_IDs__c":"1290860006; 771233008; C0334121; C6481; D006104; DOID:0050905; MEDGEN:137723; MONDO:0015798; ORPHA:178342","USA_Estimate__c":null,"No_of_Specialist_Tagsa__c":1,"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":4,"Disease_Characteristics_Score__c":2,"No_of_Age_at_Onset__c":0,"Description_Source__c":"MONDO:0015798","Disease_Description__c":"A multinodular intermediate fibroblastic neoplasm that arises from soft tissue or viscera, in children and young adults. It is characterized by the presence of spindle-shaped fibroblasts and myofibroblasts, and a chronic inflammatory infiltrate composed of eosinophils, lymphocytes, and plasma cells.","GARD_Name__c":"Inflammatory myofibroblastic tumor","GARD_Synonym__c":"imt; inflammatory fibrosarcoma; inflammatory myofibroblastic neoplasm; inflammatory pseudotumor","Curated_Disease_Description_Source__c":"GARD:0007146","Curated_Disease_Description__c":"An Inflammatory myofibroblastic tumor (IMT) is an uncommon, usually benign (non-cancerous) tumor made up of cells called myofibroblastic spindle cells. It usually develops in children or young adults, but can affect people of any age. An IMT can occur in almost any part of the body but is most commonly found in the lung, orbit (eye socket), peritoneum (lining of the abdominal cavity and internal organs), and mesentery. Signs and symptoms vary depending on the site of the tumor. Some people with an IMT are asymptomatic, while others may have nonspecific respiratory symptoms, fever, or pain. IMTs may recur, and become locally invasive and/or spread (metastasize) to other parts of the body. However, malignant (cancerous) IMTs are rare. The underlying cause of IMTs is poorly understood. Some cases have been linked to translocations involving the ALK gene.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":null,"Age_at_Onset_Snippet_Text__c":null,"SourceID__c":"ORPHA:178342","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Grouping","MONDO_ID__c":"MONDO:0015798","ORPHANET_ID__c":"ORPHA:178342","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Tumor miofibroblástico inflamatorio","Spanish_Description_Source__c":null,"Spanish_Description__c":null,"Spanish_Disease_Name__c":"tumor miofibroblástico inflamatorio","Spanish_GARD_Synonym__c":null,"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":"An Inflammatory myofibroblastic tumor (IMT) is an uncommon, usually benign (non-cancerous) tumor made up of cells called myofibroblastic spindle cells. It usually develops in children or young adults, but can affect people of any age. An IMT can occur in almost any part of the body but is most commonly found in the lung, orbit (eye socket), peritoneum (lining of the abdominal cavity and internal organs), and mesentery. Signs and symptoms vary depending on the site of the tumor. Some people with an IMT are asymptomatic, while others may have nonspecific respiratory symptoms, fever, or pain. IMTs may recur, and become locally invasive and/or spread (metastasize) to other parts of the body. However, malignant (cancerous) IMTs are rare. The underlying cause of IMTs is poorly understood. Some cases have been linked to translocations involving the ALK gene.","Curated_Disease_Description_Source__c":"GARD:0007146","GARD_Synonym__c":"imt; inflammatory fibrosarcoma; inflammatory myofibroblastic neoplasm; inflammatory pseudotumor","Name":"Inflammatory myofibroblastic tumor","estimateUsa":""}],"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"}],"External_Identifier_Disease__c":[{"URL__c":"https://raresource.nih.gov/diseases/filter/0007146","Source__c":"RareSource"},{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C6481","Source__c":"C0334121; MONDO:0015798","Xref__c":"C6481"},{"URL__c":"https://www.ebi.ac.uk/ols4/ontologies/doid/classes?obo_id=DOID%3A0050905","Source__c":"MONDO:0015798","Xref__c":"DOID:0050905"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=137723","Source__c":"C0334121","Xref__c":"MEDGEN:137723"},{"URL__c":"https://www.ncbi.nlm.nih.gov/mesh/C006104","Source__c":"MONDO:0015798","Xref__c":"D006104"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C0334121","Source__c":"C0334121","Xref__c":"C0334121"},{"URL__c":"https://www.orpha.net/en/disease/detail/178342","Source__c":"C0334121; MONDO:0015798; ORPHA:178342","Xref__c":"ORPHA:178342"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0015798","Source__c":"GARD:0007146","Xref__c":"MONDO:0015798"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=1290860006","Source__c":"C0334121","Xref__c":"1290860006"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=771233008","Source__c":"C0334121","Xref__c":"771233008"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=35073002","Source__c":"C0334121","Xref__c":"35073002"}],"tags":{"Specialist":["Cancer - Oncologist"],"Disease Category":["Cancer"]},"synonyms":["imt"," inflammatory fibrosarcoma"," inflammatory myofibroblastic neoplasm"," inflammatory pseudotumor"]}