Fibrolamellar hepatocellular carcinoma
diseaseOn this page
Also known as eosinophilic glassy cell hepatomaeosinophilic hepatocellular carcinoma with lamellar fibrosisFHCCfibrolamellar cancerfibrolamellar carcinomafibrolamellar carcinoma of liver cellsfibrolamellar carcinoma of the liver cellsfibrolamellar hepatocarcinomafibrolamellar oncocytic hepatomafibrolamellar variant of hepatocellular carcinomaFL-HCCFLChepatocellular carcinoma (fibrolamellar variant)hepatocellular carcinoma with increased stromal fibrosishepatocellular carcinoma, fibrolamellarhepatocellular fibrolamellar carcinomaliver cell fibrolamellar carcinomaoncocytic hepatocellular tumoroncocytic hepatocellular tumour
Summary
Fibrolamellar hepatocellular carcinoma (MONDO:0006210) is a cancer and 20 clinical trials. Top therapeutic interventions include 2-mercaptoethanesulfonic acid, irinotecan, and cisplatin. A subtype of hepatocellular carcinoma — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Classification: Cancer
- Prevalence: <1 / 1 000 000 (Europe) [Orphanet-validated]
- Clinical trials: 20
Clinical features
Epidemiology
Prevalence records
22 prevalence record(s), Orphanet, top 20 (validated / broadest geography first):
| Type | Class | Value | Geography | Validation |
|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.025 | Europe | Validated |
| Annual incidence | <1 / 1 000 000 | 0.02 | United States | Validated |
| Annual incidence | <1 / 1 000 000 | 0.009 | Austria | Validated |
| Annual incidence | <1 / 1 000 000 | 0.019 | Belgium | Validated |
| Annual incidence | <1 / 1 000 000 | 0.029 | Bulgaria | Validated |
| Annual incidence | <1 / 1 000 000 | 0.029 | Czech Republic | Validated |
| Annual incidence | <1 / 1 000 000 | 0.018 | Estonia | Validated |
| Annual incidence | <1 / 1 000 000 | 0.002 | Finland | Validated |
| Annual incidence | <1 / 1 000 000 | 0.031 | France | Validated |
| Annual incidence | <1 / 1 000 000 | 0.019 | Germany | Validated |
| Annual incidence | <1 / 1 000 000 | 0.043 | Iceland | Validated |
| Annual incidence | <1 / 1 000 000 | 0.022 | Ireland | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.13 | Italy | Validated |
| Annual incidence | <1 / 1 000 000 | 0.022 | Latvia | Validated |
| Annual incidence | <1 / 1 000 000 | 0.003 | Norway | Validated |
| Annual incidence | <1 / 1 000 000 | 0.005 | Poland | Validated |
| Annual incidence | <1 / 1 000 000 | 0.006 | Portugal | Validated |
| Annual incidence | <1 / 1 000 000 | 0.008 | Slovakia | Validated |
| Annual incidence | <1 / 1 000 000 | 0.009 | Spain | Validated |
| Annual incidence | <1 / 1 000 000 | 0.017 | Switzerland | Validated |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | fibrolamellar hepatocellular carcinoma |
| Mondo ID | MONDO:0006210 |
| EFO | EFO:1000256 |
| MeSH | C537258 |
| Orphanet | 401920 |
| DOID | DOID:5015 |
| NCIT | C4131 |
| SNOMED CT | 253018005 |
| UMLS | C0334287 |
| MedGen | 83122 |
| GARD | 0021704 |
| Is cancer (heuristic) | yes |
Also known as: eosinophilic glassy cell hepatoma · eosinophilic hepatocellular carcinoma with lamellar fibrosis · FHCC · fibrolamellar cancer · fibrolamellar carcinoma · fibrolamellar carcinoma of liver cells · fibrolamellar carcinoma of the liver cells · fibrolamellar hepatocarcinoma · fibrolamellar hepatocellular carcinoma · fibrolamellar oncocytic hepatoma · fibrolamellar variant of hepatocellular carcinoma · FL-HCC · FLC · hepatocellular carcinoma (fibrolamellar variant) · hepatocellular carcinoma with increased stromal fibrosis · hepatocellular carcinoma, fibrolamellar · hepatocellular fibrolamellar carcinoma · liver cell fibrolamellar carcinoma · oncocytic hepatocellular tumor · oncocytic hepatocellular tumour (+2 more)
Disease family
This is a subtype of hepatocellular carcinoma. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.
Classification path: disease › human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › cancer › carcinoma › adenocarcinoma › hepatocellular carcinoma › fibrolamellar hepatocellular carcinoma
Related subtypes (7): hepatocellular clear cell carcinoma, aflatoxin-related hepatocellular carcinoma, sclerosing hepatic carcinoma, liver diffuse large B-cell lymphoma, adult hepatocellular carcinoma, pediatric hepatocellular carcinoma, hepatoblastoma
Subtypes (1): mixed fibrolamellar hepatocellular carcinoma
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
No tiered GWAS variants or ClinVar records for this disease.
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
No druggable-target or therapeutic data for this disease’s cohort.
Clinical trials & evidence
Clinical trials
Clinical trials: 20.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE2 | 6 |
| PHASE1/PHASE2 | 5 |
| PHASE1 | 5 |
| Not specified | 3 |
| PHASE3 | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT03533582 | PHASE3 | ACTIVE_NOT_RECRUITING | Cisplatin and Combination Chemotherapy in Treating Children and Young Adults With Hepatoblastoma or Liver Cancer After Surgery |
| NCT04134559 | PHASE2 | RECRUITING | Checkpoint Inhibition In Pediatric Hepatocellular Carcinoma |
| NCT05468359 | PHASE1/PHASE2 | RECRUITING | Safety and Efficacy of Cyclophosphamide, Sorafenib, Bevacizumab, and Atezolizumab in Pediatric Solid Tumor Patients |
| NCT06027086 | PHASE1/PHASE2 | RECRUITING | DRP-104 (Glutamine Antagonist) in Combination With Durvalumab in Patients With Advanced Stage Fibrolamellar Carcinoma (FLC) |
| NCT06521567 | PHASE1/PHASE2 | ACTIVE_NOT_RECRUITING | A Study of Cobolimab Plus Dostarlimab in Pediatric and Young Adult Participants With Cancer |
| NCT06620302 | PHASE1/PHASE2 | RECRUITING | Testing the Addition of an Anti-cancer Drug, DT2216, to the Usual Chemotherapy Treatment for Relapsed or Refractory Solid Tumors and Fibrolamellar Carcinoma |
| NCT06638931 | PHASE2 | RECRUITING | Agnostic Therapy in Rare Solid Tumors |
| NCT01215565 | PHASE2 | TERMINATED | Study of Sunitinib in Patients With Advanced/Inoperable Fibrolamellar Carcinoma |
| NCT01642186 | PHASE2 | COMPLETED | 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) |
| NCT02234986 | PHASE2 | COMPLETED | A Multi-center, Open-label Study of Oral ENMD-2076 for the Treatment of Patients With Advanced Fibrolamellar Carcinoma |
| NCT02702960 | PHASE2 | WITHDRAWN | Sequential, Related Donor Partial Liver Transplantation Followed by Bone Marrow Transplantation for Hepatocellular Carcinoma (HCC) |
| NCT05603572 | PHASE1/PHASE2 | SUSPENDED | KAT-101 in Subjects With Hepatocellular Carcinoma (HCC) |
| NCT03860272 | PHASE1 | ACTIVE_NOT_RECRUITING | Fc-Engineered Anti-CTLA-4 Monoclonal Antibody in Advanced Cancer |
| NCT04248569 | PHASE1 | RECRUITING | DNAJB1-PRKACA Fusion Kinase Peptide Vaccine Combined With Nivolumab and Ipilimumab for Patients With Fibrolamellar Hepatocellular Carcinoma |
| NCT05937295 | PHASE1 | RECRUITING | FusionVAC22_01: Fusion Transcript-based Peptide Vaccine Combined with Immune Checkpoint Inhibition |
| NCT06789198 | PHASE1 | RECRUITING | Peptide Vaccine for Fibrolamellar Hepatocellular Carcinoma Patients and Other Tumor Entities Carrying the Driver Fusion DNAJB1-PRKACA |
| NCT02159989 | PHASE1 | COMPLETED | Sapanisertib and Ziv-Aflibercept in Treating Patients With Recurrent Solid Tumors That Are Metastatic or Cannot Be Removed by Surgery |
| NCT06541652 | Not specified | RECRUITING | A French Multicenter Observational Retrospective Study of Rare Primary Liver Cancers |
| NCT04874519 | Not specified | COMPLETED | Registry to Collect Health Information Related to Fibrolamellar Cancer |
| NCT05681949 | Not specified | UNKNOWN | Therapeutic Assistance and Decision-making Algorithms in Hepatobiliary Tumor Boards |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| 2-MERCAPTOETHANESULFONIC ACID | 4 | 2 |
| IRINOTECAN | 4 | 2 |
| CISPLATIN | 4 | 1 |
| DOSTARLIMAB | 4 | 1 |
| SORAFENIB | 4 | 1 |
| SUNITINIB | 4 | 1 |
| BALSTILIMAB | 3 | 1 |
| BOTENSILIMAB | 3 | 1 |
| COBOLIMAB | 2 | 1 |
| ENMD-2076 | 2 | 1 |
| SAPANISERTIB | 2 | 1 |
| SIRPIGLENASTAT | 1 | 1 |
| CHEMBL4073387 | 0 | 1 |
| CHEMBL3962632 | 0 | 1 |
| CHEMBL3991933 | 0 | 1 |