{"Name":"Fibrolamellar hepatocellular carcinoma","DiseaseID__c":"GARD:0021704","id":21704,"encodedName":"fibrolamellar-hepatocellular-carcinoma","IsDeleted":false,"Disease_Name_Full__c":"Fibrolamellar hepatocellular carcinoma","Xref_IDs__c":"253018005; 423022163; C0334287; C4131; C537258; DOID:5015; MEDGEN:83122; MONDO:0006210; ORPHA:401920","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":1,"World_Estimate__c":"1 to 8,000","No_of_HRSA_records__c":0,"Evidence_Based_Score__c":1,"No_of_Disease_Descriptions__c":2,"Disease_Characteristics_Score__c":4,"No_of_Age_at_Onset__c":2,"Description_Source__c":"MONDO:0006210","Disease_Description__c":"A distinctive type of liver cell carcinoma that arises in non-cirrhotic livers and is seen predominantly in young patients. The tumor cells are polygonal and deeply eosinophilic, and are embedded in a fibrous stroma. The prognosis is similar to classical hepatocellular carcinoma that arises in non-cirrhotic livers, and better than hepatocellular carcinoma that arises in cirrhotic livers.","GARD_Name__c":"Fibrolamellar hepatocellular carcinoma","GARD_Synonym__c":"fhcc; fibrolamellar cancer; fibrolamellar carcinoma; fibrolamellar carcinoma of liver cells; fibrolamellar carcinoma of the liver cells; fibrolamellar hepatocarcinoma; flc; hepatocellular carcinoma, fibrolamellar; hepatocellular fibrolamellar carcinoma; liver cell fibrolamellar carcinoma; oncocytic hepatocellular tumor; oncocytic hepatocellular tumour; polygonal cell type hepatocellular carcinoma with fibrous stroma","Curated_Disease_Description_Source__c":"MONDO:0006210","Curated_Disease_Description__c":"A rare variant of hepatocellular carcinoma (HCC) presenting in adolescents or young adults with no underlying liver disease. Clinical presentation is non specific, with abdominal mass, abdominal discomfort or pain, fatigue and weight loss. Patients can also be asymptomatic. HCC markers (alpha fetoprotein) are normal. Fibrolamellar HCC presents as a unique, well-delimited mass at imagery and a biopsy confirms the diagnosis, showing well-differentiated tumor cells surrounded by thick collagen bands.","Curated_USA_Estimate_Source__c":null,"Curated_USA_Estimate__c":"1,000","Age_at_Onset_Snippet_Text__c":"as a Teenager and as an Adult","SourceID__c":"ORPHA:401920","Deprecated__c":"No","Disease_Concept_Type__c":"Rare Disease Grouping","MONDO_ID__c":"MONDO:0006210","ORPHANET_ID__c":"ORPHA:401920","Replaced_By_ID__c":null,"Display_Spanish_Disease_Name__c":"Carcinoma hepatocelular fibrolamelar","Spanish_Description_Source__c":null,"Spanish_Description__c":null,"Spanish_Disease_Name__c":"carcinoma hepatocelular fibrolamelar","Spanish_GARD_Synonym__c":"fhcc; hepatocarcinoma fibromelar","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 rare variant of hepatocellular carcinoma (HCC) presenting in adolescents or young adults with no underlying liver disease. Clinical presentation is non specific, with abdominal mass, abdominal discomfort or pain, fatigue and weight loss. Patients can also be asymptomatic. HCC markers (alpha fetoprotein) are normal. Fibrolamellar HCC presents as a unique, well-delimited mass at imagery and a biopsy confirms the diagnosis, showing well-differentiated tumor cells surrounded by thick collagen bands.","Curated_Disease_Description_Source__c":"MONDO:0006210","GARD_Synonym__c":"fhcc; fibrolamellar cancer; fibrolamellar carcinoma; fibrolamellar carcinoma of liver cells; fibrolamellar carcinoma of the liver cells; fibrolamellar hepatocarcinoma; flc; hepatocellular carcinoma, fibrolamellar; hepatocellular fibrolamellar carcinoma; liver cell fibrolamellar carcinoma; oncocytic hepatocellular tumor; oncocytic hepatocellular tumour; polygonal cell type hepatocellular carcinoma with fibrous stroma","Name":"Fibrolamellar hepatocellular carcinoma","Curated_USA_Estimate__c":"1,000","estimateUsa":"1,000"}],"Organization_Supported_Diseases__c":[{"Account_Name__c":"The Fibrolamellar Registry","Website__c":"https://fibroregistry.org/"},{"Account_Name__c":"American Cancer Society","Website__c":"https://www.cancer.org"},{"Account_Name__c":"Fibrolamellar Cancer Foundation","Website__c":"https://fibrofoundation.org/"},{"Account_Name__c":"American Liver Foundation","Website__c":"https://liverfoundation.org/"},{"Account_Name__c":"Blue Faery The Adrienne Wilson Liver Cancer Association","Website__c":"https://www.bluefaery.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":"Gastroenterology","Tag_Category__c":"Disease Category;Specialist","category_description":"Gastrointestinal diseases, or digestive diseases, affect the esophagus, stomach, small intestine, large intestine, liver, gallbladder, or pancreas.","curated_tag_name":"Gastrointestinal diseases"},{"Tag_Name__c":"Pediatrics","Tag_Category__c":"Specialist"}],"Age_At_Onset__c":[{"Age_At_Onset__c":"Adult","Provided_By__c":"ORPHA:401920"},{"Age_At_Onset__c":"Adolescent","Provided_By__c":"ORPHA:401920"}],"External_Identifier_Disease__c":[{"URL__c":"https://evsexplore.semantics.cancer.gov/evsexplore/concept/ncit/C4131","Source__c":"C0334287; MONDO:0006210","Xref__c":"C4131"},{"URL__c":"https://uts.nlm.nih.gov/uts/umls/concept/C0334287","Source__c":"C0334287","Xref__c":"C0334287"},{"URL__c":"https://www.orpha.net/en/disease/detail/401920","Source__c":"C0334287; MONDO:0006210; ORPHA:401920","Xref__c":"ORPHA:401920"},{"URL__c":"https://www.ebi.ac.uk/ols4/ontologies/doid/classes?obo_id=DOID%3A5015","Source__c":"MONDO:0006210","Xref__c":"DOID:5015"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=253018005","Source__c":"C0334287; MONDO:0006210","Xref__c":"253018005"},{"URL__c":"https://www.ncbi.nlm.nih.gov/medgen/?term=83122","Source__c":"C0334287","Xref__c":"MEDGEN:83122"},{"URL__c":"https://www.ncbi.nlm.nih.gov/mesh/C537258","Source__c":"MONDO:0006210","Xref__c":"C537258"},{"URL__c":"http://purl.obolibrary.org/obo/MONDO_0006210","Source__c":"GARD:0021704","Xref__c":"MONDO:0006210"},{"URL__c":"https://browser.ihtsdotools.org/?perspective=full&conceptId1=15619004","Source__c":"C0334287","Xref__c":"15619004"},{"URL__c":"https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022163","Xref__c":"423022163"}],"tags":{"Specialist":["Cancer - Oncologist","Gastroenterology","Pediatrics"],"Disease Category":["Cancer","Gastroenterology"]},"synonyms":["fhcc"," fibrolamellar cancer"," fibrolamellar carcinoma"," fibrolamellar carcinoma of liver cells"," fibrolamellar carcinoma of the liver cells"," fibrolamellar hepatocarcinoma"," flc"," hepatocellular carcinoma, fibrolamellar"," hepatocellular fibrolamellar carcinoma"," liver cell fibrolamellar carcinoma"," oncocytic hepatocellular tumor"," oncocytic hepatocellular tumour"," polygonal cell type hepatocellular carcinoma with fibrous stroma"]}