{"Name":"Littoral cell angioma","DiseaseID__c":"GARD:0009714","id":9714,"encodedName":"littoral-cell-angioma","IsDeleted":false,"Disease_Name_Full__c":"Littoral cell angioma","Xref_IDs__c":"418040002; C1627365; C200524; C537031; MEDGEN:351507; MONDO:0023650","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":3,"Disease_Characteristics_Score__c":2,"No_of_Age_at_Onset__c":0,"Description_Source__c":"MONDO:0023650","Disease_Description__c":"Littoral cell angioma is a rare primary vascular neoplasm of the spleen, composed of littoral cells that line the splenic sinuses of the red pulp. It was thought to be a benign, incidental lesion. However, many recent reports have described it to be a malignant lesion with congenital and immunological associations. The definitive diagnosis can only be made after histology and immunohistochemistry studies.","GARD_Name__c":"Littoral cell angioma","GARD_Synonym__c":"epithelioid hemangioendothelioma of spleen; lca; littoral cell angioma of the spleen","Curated_Disease_Description_Source__c":"GARD:0009714","Curated_Disease_Description__c":"Littoral cell angioma of the spleen (LCA) is a very rare, vascular tumor of the spleen. Most reported cases have been non-cancerous (benign). In many cases, LCA does not cause any symptoms. When symptoms occur, they may include abdominal pain, an enlarged spleen (splenomegaly), anemia, or thrombocytopenia. It may be discovered due to abdominal pain or by accident (incidentally) when having a physical exam or during tests performed for other reasons. LCA affects men and women equally and can occur at any age, but it mostly occurs in middle-aged adults and occasionally in children. LCA is difficult to diagnose with imaging studies because of its similar appearance to other types of benign and malignant tumors.. The diagnosis is usually confirmed by biopsy or pathological evaluation of removed tumor tissue.","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:0023650","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":"Littoral cell angioma of the spleen (LCA) is a very rare, vascular tumor of the spleen. Most reported cases have been non-cancerous (benign). In many cases, LCA does not cause any symptoms. When symptoms occur, they may include abdominal pain, an enlarged spleen (splenomegaly), anemia, or thrombocytopenia. It may be discovered due to abdominal pain or by accident (incidentally) when having a physical exam or during tests performed for other reasons. LCA affects men and women equally and can occur at any age, but it mostly occurs in middle-aged adults and occasionally in children. LCA is difficult to diagnose with imaging studies because of its similar appearance to other types of benign and malignant tumors.. The diagnosis is usually confirmed by biopsy or pathological evaluation of removed tumor tissue.","Curated_Disease_Description_Source__c":"GARD:0009714","GARD_Synonym__c":"epithelioid hemangioendothelioma of spleen; lca; littoral cell angioma of the spleen","Name":"Littoral cell angioma","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"},{"Tag_Name__c":"Vascular Medicine","Tag_Category__c":"Specialist","curated_tag_name":"Vascular diseases"}],"External_Identifier_Disease__c":[{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=351507","Source__c":"C1627365","Xref__c":"MEDGEN:351507"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=418040002","Source__c":"C1627365; MONDO:0023650","Xref__c":"418040002"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C1627365","Source__c":"C1627365","Xref__c":"C1627365"},{"URL__c":"https://www.ncbi.nlm.nih.gov/mesh/C537031","Source__c":"MONDO:0023650","Xref__c":"C537031"},{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C200524","Source__c":"C1627365; MONDO:0023650","Xref__c":"C200524"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0023650","Source__c":"GARD:0009714","Xref__c":"MONDO:0023650"}],"tags":{"Disease Category":["Cancer"],"Specialist":["Vascular Medicine"]},"synonyms":["epithelioid hemangioendothelioma of spleen"," lca"," littoral cell angioma of the spleen"]}