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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 |
| 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 | Covered Countries | Other Countries |
|---|---|---|---|---|---|---|
| NCT01396408 | Phase II | Temsirolimus Sunitinib | A Phase II Study of Sunitinib or Temsirolimus in Patients With Advanced Rare Tumours | Active, not recruiting | CAN | 0 |
| 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 | USA | 0 |
| 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 | USA | 0 |
| NCT04338685 | Phase I | RO7119929 + Tocilizumab | A Study Evaluating Safety, Pharmacokinetics, Pharmacodynamics, And Clinical Activity Of RO7119929 (TLR7 Agonist) In Participants With Unresectable Advanced Or Metastatic Hepatocellular Carcinoma, Biliary Tract Cancer, Or Solid Tumors With Hepatic Metastases | Completed | USA | ESP | DNK | 3 |
| NCT04380545 | Phase Ib/II | Fluorouracil + Nivolumab + Novaferon | Nivolumab, Fluorouracil, and Interferon Alpha 2B for the Treatment of Unresectable Fibrolamellar Cancer | Recruiting | USA | 0 |
| NCT05468359 | Phase Ib/II | Atezolizumab + Bevacizumab + Cyclophosphamide + Sorafenib | Safety and Efficacy of Cyclophosphamide, Sorafenib, Bevacizumab, and Atezolizumab in Pediatric Solid Tumor Patients | Suspended | USA | 0 |
| NCT05937295 | Phase I | Fusion-VAC-XS15 Atezolizumab + Fusion-VAC-XS15 | FusionVAC22_01: Fusion Transcript-based Peptide Vaccine Combined With Immune Checkpoint Inhibition (FusionVAC22) | Recruiting | DEU | 0 |
| NCT06027086 | Phase Ib/II | DRP-104 + Durvalumab | DRP-104 (Glutamine Antagonist) in Combination With Durvalumab in Patients With Advanced Stage Fibrolamellar Carcinoma (FLC) | Recruiting | USA | 0 |
| NCT06521567 | Phase Ib/II | Cobolimab + Dostarlimab-gxly | A Study of Cobolimab Plus Dostarlimab in Pediatric and Young Adult Participants With Cancer | Active, not recruiting | USA | ITA | FRA | ESP | DNK | CZE | 0 |
| NCT06620302 | Phase Ib/II | DT2216 + Irinotecan | Testing the Addition of an Anti-cancer Drug, DT2216, to the Usual Chemotherapy Treatment for Relapsed or Refractory Solid Tumors and Fibrolamellar Carcinoma | Recruiting | USA | 0 |
| NCT06638931 | Phase II | Nivolumab | Agnostic Therapy in Rare Solid Tumors (ANTARES) | Active, not recruiting | BRA | 0 |
| NCT06789198 | Phase I | Fusion-VAC-XS15 | Fusion Transcript-based Peptide Vaccine for Fibrolamellar Hepatocellular Carcinoma Patients and Other Tumor Entities Carrying the Oncogenic Driver Fusion DNAJB1-PRKACA (FusionVAC22_02) | Not yet recruiting | DEU | 0 |