Disease Detail


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ID DOID:5015
Name fibrolamellar carcinoma
Definition A hepatocellular carcinoma characterized microscopically by laminated fibrous layers interspersed between the tumour cells. The polygonal, deeply eosinophilic tumor cells arise in non-cirrhotic livers.
Source DiseaseOntology.org
Alt Ids
Path disease disease of cellular proliferation cancer organ system cancer gastrointestinal system cancer hepatobiliary system cancer liver cancer liver carcinoma hepatocellular carcinoma fibrolamellar carcinoma


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Molecular Profile Therapy Indication/Tumor Type Response Type Profile Response Detail
FBXW7 E192A Sirolimus fibrolamellar carcinoma predicted - sensitive detail...
Clinical Trial Phase Therapies Title Recruitment Status
NCT01396408 Phase II Temsirolimus Sunitinib A Phase II Study of Sunitinib or Temsirolimus in Patients With Advanced Rare Tumours Active, not recruiting
NCT02234986 Phase II ENMD-2076 A Multi-center, Open-label Study of Oral ENMD-2076 for the Treatment of Patients With Advanced Fibrolamellar Carcinoma Completed
NCT04248569 Phase I DNAJB1-PRKACA peptide vaccine + Ipilimumab + Nivolumab + Poly ICLC DNAJB1-PRKACA Fusion Kinase Peptide Vaccine Combined With Nivolumab and Ipilimumab for Patients With Fibrolamellar Hepatocellular Carcinoma Recruiting
NCT04380545 Phase Ib/II Fluorouracil + Nivolumab + Novaferon Nivolumab, Fluorouracil, and Interferon Alpha 2B for the Treatment of Unresectable Fibrolamellar Cancer Not yet recruiting