Disease · MONDO_0006210

fibrolamellar hepatocellular carcinoma

6 sourcesdiseases-2026-09-18-0.1.0

Cite this record

ConductScience Library. fibrolamellar hepatocellular carcinoma. Diseases, record MONDO_0006210. Release diseases-2026-09-18-0.1.0, 18 Sept 2026. https://conductscience.org/library/diseases/MONDO_0006210

On this page

Disease

Names and IDs as Open Targets gives them. Its IDs in MeSH, Orphanet, the Disease Ontology and the NCI Thesaurus are those Open Targets states for it.

Name: fibrolamellar hepatocellular carcinoma; Open Targets
Open Targets ID: MONDO_0006210; Open Targets
Other names: FHCC, FLC, fibrolamellar cancer, fibrolamellar carcinoma, fibrolamellar carcinoma of liver cells, fibrolamellar carcinoma of the liver cells, fibrolamellar hepatocarcinoma, hepatocellular carcinoma, fibrolamellar, hepatocellular fibrolamellar carcinoma, liver cell fibrolamellar carcinoma, oncocytic hepatocellular tumor, oncocytic hepatocellular tumour, polygonal cell type hepatocellular carcinoma with fibrous Stroma; Open Targets
Disease Ontology: DOID:5015; Disease Ontology
MeSH: C537258; MeSH
NCI Thesaurus: C4131; NCI Thesaurus
Orphanet: ORPHA:401920; Orphanet
Highest stage of its drugs: Phase 2, for 6 of its 22 drugs, from ClinicalTrials.gov; Open Targets

Drugs22

The drugs Open Targets' clinical reports study for the disease, the highest stage first, then the most trial reports. The stage is Open Targets', the highest any of its sources reports for the pair, and each row names the sources that report it.

Drug	ChEMBL ID	Type	Open Targets' stage	Stage reported by	Reports	Of them trials	Trials shown here	Source
7-ETHYL-10-HYDROXYCAMPTOTHECIN	CHEMBL837	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
ENMD-2076	CHEMBL482968	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
EVEROLIMUS	CHEMBL1908360	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
LETROZOLE	CHEMBL1444	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
LEUPROLIDE	CHEMBL1201199	Protein	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
VANCOMYCIN	CHEMBL262777	Small molecule	Phase 2	ClinicalTrials.gov	1	1	1	Drugs and targets
NIVOLUMAB	CHEMBL2108738	Antibody	Phase 1/2	ClinicalTrials.gov	3	3	3	Drugs and targets
ATEZOLIZUMAB	CHEMBL3707227	Antibody	Phase 1/2	ClinicalTrials.gov	2	2	2	Drugs and targets
BEVACIZUMAB	CHEMBL1201583	Antibody	Phase 1/2	ClinicalTrials.gov	1	1	1	Drugs and targets
CYCLOPHOSPHAMIDE	CHEMBL1200796	Small molecule	Phase 1/2	ClinicalTrials.gov	1	1	1	Drugs and targets

1 to 10 of 22

See all 22 drugs

Targets24

The targets ChEMBL's mechanism records give for the disease's drugs, through Open Targets, the most drugs first. A mechanism record says what a drug acts on, not that the target causes the disease.

Target	Protein	Its drugs that act on it	Mechanisms	Highest stage of those drugs	Gene in Genes	Source
IFNAR1	Interferon alpha/beta receptor	3	Interferon alpha/beta receptor agonist; Interferon alpha/beta receptor positive modulator	Phase 1/2	https://conductscience.org/library/genes/3454	Drugs and targets
IFNAR2	Interferon alpha/beta receptor	3	Interferon alpha/beta receptor agonist; Interferon alpha/beta receptor positive modulator	Phase 1/2	https://conductscience.org/library/genes/3455	Drugs and targets
FLT3	Receptor-type tyrosine-protein kinase FLT3	2	Tyrosine-protein kinase receptor FLT3 inhibitor	Phase 2	https://conductscience.org/library/genes/2322	Drugs and targets
KDR	Vascular endothelial growth factor receptor	2	Vascular endothelial growth factor receptor 2 inhibitor; Vascular endothelial growth factor receptor inhibitor	Phase 2	https://conductscience.org/library/genes/3791	Drugs and targets
KIT	Mast/stem cell growth factor receptor Kit	2	Stem cell growth factor receptor inhibitor	Phase 2	https://conductscience.org/library/genes/3815	Drugs and targets
PDCD1	Programmed cell death protein 1	2	Programmed cell death protein 1 antagonist; Programmed cell death protein 1 inhibitor	Phase 1/2	https://conductscience.org/library/genes/5133	Drugs and targets
TOP1	DNA topoisomerase 1	2	DNA topoisomerase I inhibitor	Phase 2	https://conductscience.org/library/genes/7150	Drugs and targets
AURKA	Aurora kinase A	1	Serine/threonine-protein kinase Aurora-A inhibitor	Phase 2	https://conductscience.org/library/genes/6790	Drugs and targets
AURKB	Aurora kinase B	1	Serine/threonine-protein kinase Aurora-B inhibitor	Phase 2	https://conductscience.org/library/genes/9212	Drugs and targets
BRAF	Serine/threonine-protein kinase B-raf	1	Serine/threonine-protein kinase B-raf inhibitor	Phase 1/2	https://conductscience.org/library/genes/673	Drugs and targets

1 to 10 of 24

See all 24 targets

Trials11

Trials an Open Targets clinical report links to the disease, posted results first, then the largest. A trial is shown when the release's checks confirm its link to the disease and Drugs and targets shows its link to one of the disease's drugs.

Trial	Title	Phase	Participants	Results first posted	Conditions	Drugs	Linked by	Source
NCT02234986	A Multi-center, Open-label Study of Oral ENMD-2076 for the Treatment of Patients With Advanced Fibrolamellar Carcinoma	Phase 2	35	2025-04-18	Advanced Adult Hepatocellular Carcinoma, Advanced Fibrolamellar Carcinoma	ENMD-2076	Its record names the disease	ClinicalTrials.gov
NCT01642186	Study of (1) Everolimus, (2) Estrogen Deprivation Therapy (EDT) With Leuprolide + Letrozole and (3) Everolimus + EDT in Patients With Unresectable Fibrolamellar Hepatocellular Carcinoma (FLL-HCC)	Phase 2	28	2022-12-29	Fibrolamellar Carcinoma, Fibrolamellar Liver Cancer	EVEROLIMUS, LETROZOLE, LEUPROLIDE	Its record names the disease	ClinicalTrials.gov
NCT03860272	Fc-Engineered Anti-CTLA-4 Monoclonal Antibody in Advanced Cancer	Phase 1	499		Advanced Cancer, Angiosarcoma, Colorectal Cancer Without Liver Metastases, Endometrial Cancer, Fibrolamellar Carcinoma, Non-small-cell Lung Cancer, Ovarian Cancer, Prostate Cancer	BALSTILIMAB, BOTENSILIMAB	Its record names the disease	ClinicalTrials.gov
NCT06620302	Testing the Addition of an Anti-cancer Drug, DT2216, to the Usual Chemotherapy Treatment for Relapsed or Refractory Solid Tumors and Fibrolamellar Carcinoma	Phase 1/2	81		Childhood Fibrolamellar Carcinoma, Recurrent Childhood Fibrolamellar Carcinoma, Recurrent Childhood Malignant Solid Neoplasm, Recurrent Fibrolamellar Carcinoma, Recurrent Malignant Solid Neoplasm, Refractory Childhood Fibrolamellar Carcinoma, Refractory Childhood Malignant Solid Neoplasm, Refractory Fibrolamellar Carcinoma, Refractory Malignant Solid Neoplasm	DT-2216, IRINOTECAN	Its record names the disease	ClinicalTrials.gov
NCT06986811	CEB-01 in Pediatrics With Locally Resectable Abdominal Tumors	Phase 2	80		Locally Resectable Pediatrics Tumors	7-ETHYL-10-HYDROXYCAMPTOTHECIN	Its record names the disease	ClinicalTrials.gov
NCT05468359	Safety and Efficacy of Cyclophosphamide, Sorafenib, Bevacizumab, and Atezolizumab in Pediatric Solid Tumor Patients	Phase 1/2	64		Refractory Solid Tumor, Hepatocellular Carcinoma, Malignant Solid Tumor, Pediatric Cancer, Pediatric Solid Tumor, Fibrolamellar Carcinoma	ATEZOLIZUMAB, BEVACIZUMAB, CYCLOPHOSPHAMIDE, SORAFENIB	Its record names the disease	ClinicalTrials.gov
NCT04248569	DNAJB1-PRKACA Fusion Kinase Peptide Vaccine Combined With Nivolumab and Ipilimumab for Patients With Fibrolamellar Hepatocellular Carcinoma	Phase 1	56		Fibrolamellar Hepatocellular Carcinoma (FLC)	IPILIMUMAB, NIVOLUMAB	Its record names the disease	ClinicalTrials.gov
NCT07430202	A DNAJB1-PRKACA Fusion Kinase Peptide Vaccine Combined With Glutamine Antagonist DRP-104, Nivolumab, and Ipilimumab in Patients With Advanced Stage Fibrolamellar Carcinoma (FLC)	Phase 1	27		Liver Cancer (Fibrolamellar Hepatocellular Cancer (FLC))	IPILIMUMAB, NIVOLUMAB, SIRPIGLENASTAT	Its record names the disease	ClinicalTrials.gov
NCT05937295	FusionVAC22_01: Fusion Transcript-based Peptide Vaccine Combined with Immune Checkpoint Inhibition	Phase 1	20		Fibrolamellar Hepatocellular Carcinoma	ATEZOLIZUMAB	Its record names the disease	ClinicalTrials.gov
NCT04380545	Nivolumab, Fluorouracil, and Interferon Alpha 2B for the Treatment of Unresectable Fibrolamellar Cancer	Phase 1/2	4		Stage III Hepatocellular Carcinoma AJCC v8, Stage IIIA Hepatocellular Carcinoma AJCC v8, Stage IIIB Hepatocellular Carcinoma AJCC v8, Stage IV Hepatocellular Carcinoma AJCC v8, Stage IVA Hepatocellular Carcinoma AJCC v8, Stage IVB Hepatocellular Carcinoma AJCC v8, Unresectable Fibrolamellar Carcinoma	FLUOROURACIL, NIVOLUMAB	Its record names the disease	ClinicalTrials.gov

1 to 10 of 11

See all 11 trials

Comparisons in its trials0

Comparisons Trial comparisons calculated in the trials shown here, most participants first. A comparison in one of these trials does not always compare a treatment for the disease, so read the two groups.

None in this release.

Papers from its trials9

The papers the trials shown here list on ClinicalTrials.gov as their results (RESULT) or as drawn from them (DERIVED), results first, newest first.

PMID	Listed as	Trials listing it	Source
41502912	DERIVED	1	PubMed
41286513	DERIVED	1	PubMed
39869830	DERIVED	1	PubMed
38871975	DERIVED	1	PubMed
38606105	DERIVED	1	PubMed
35561331	DERIVED	1	PubMed
34272087	DERIVED	2	PubMed
32154962	DERIVED	1	PubMed
25486412	DERIVED	1	PubMed

Data dates

The date of each source this release read.

Open Targets: 2026-09-18; Open Targets
Drug links and their checks, from Drugs and targets: 2026-09-18; Drugs and targets
ClinicalTrials.gov, from Trials: 2026-11-12; Trials
Comparisons, from Trial comparisons: 2026-03-23; Trial comparisons
PubMed, for the checks: 2026-11-06; PubMed
MeSH, for the checks: 2026-11-10; MeSH

Sources 6

ClinicalTrials.gov's posted results, through CTTI's AACTFreely available. Acknowledgement requested
ChEMBL, through Open TargetsCC BY-SA 3.0
ClinicalTrials.govNo license stated
NLM's MeSHNLM's terms
Open TargetsCC0 1.0
PubMedNLM's terms

Each source's terms

ClinicalTrials.gov's posted results, through CTTI's AACTClinicalTrials.gov data via Aggregate Analysis of ClinicalTrials.gov (AACT), CTTI. CTTI makes its materials freely available and requests acknowledgement. https://aact.ctti-clinicaltrials.org/documentation/299
ChEMBL, through Open TargetsCC BY-SA 3.0, for the parts from ChEMBL. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
ClinicalTrials.govnone stated
NLM's MeSHNLM's
Open TargetsCC0 1.0. Open Targets asks that users cite its latest paper: Buniello et al. 2025, Open Targets Platform: facilitating therapeutic hypotheses building in drug discovery, Nucleic Acids Research 53(D1):D1467, PMID 39657122. Read on 8 Nov 2026 at https://platform-docs.opentargets.org/licence
PubMedNLM's terms

ClinicalTrials.gov's data carry an international copyright outside the United States: https://clinicaltrials.gov/about-site/terms-conditions.

For software

Read this disease from software.

The record and each of its lists are JSON, twenty rows a page. No key.

GET https://conductscience.org/api/diseases/v1/MONDO_0006210

GET https://conductscience.org/api/diseases/v1/MONDO_0006210/drugs?page=2

API reference For AI assistants