raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:401920

Fibrolamellar hepatocellular carcinoma

Synonyms: FHCC · Fibrolamellar hepatocarcinoma

Type
Disease
Group
Disorder
Inheritance
Not applicable
Age of onset
Adolescent, Adult
Status
active

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.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enFibrolamellar hepatocellular carcinomaFHCC · Fibrolamellar hepatocarcinoma
frCarcinome hépatocellulaire fibrolamellaireCHCF · Carcinome fibrolamellaire du foie · FHCC · Hépatocarcinome fibrolamellaire
deHepatozelluläres Karzinom, fibrolamelläresFHCC · Fibrolamelläres HCC
esCarcinoma hepatocelular fibrolamelarFHCC · Hepatocarcinoma fibromelar
itCarcinoma epatocellulare fibrolamellareEpatocarcinoma fibrolamellare · FHCC
nlFibrolamellair hepatocellulair carcinoomFHCC · Fibrolamellair hepatocarcinoom
ptCarcinoma hepatocelular fibrolamelarFHCC · Hepatocarcinoma fibrolamelar
plPostać włóknisto-blaszkowa raka wątrobowokomórkowegoFHCC · Postać włóknisto-blaszkowa raka wątroby
csFibrolamelární hepatocelulární karcinomFHCC · Fibrolamelární hepatokarcinom
ukФіброламелярна гепатоцелюлярна карциномаFHCC · Фіброламелярна гепатокарцинома
zh纤维板层肝细胞癌纤维板层型肝癌
trFibrolameller hepatoselüler karsinomFHCC · Fibrolameller hepatokarsinom

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10C22.0NTBTindexvalidated
ICD-112C12.02NTBTindexvalidated
ICD-11XH9Q35Especificvalidated
MONDO0006210Evalidated
MeSHC537258Evalidated
UMLSC0334287Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (2):

No children.

4

Genes

2 direct
GeneAssociationStatusHGNCEnsemblOMIMLocusNameSource of validation
DNAJB1Part of a fusion gene inAssessedHGNC:5270ENSG0000013200260457219p13.12DnaJ heat shock protein family (Hsp40) member B124578576[PMID]
PRKACAPart of a fusion gene inAssessedHGNC:9380ENSG0000007206260163919p13.12protein kinase cAMP-activated catalytic subunit alpha24578576[PMID]
5

Prevalence and incidence

22 records
TypeClassValueAreaQualificationValidationSource
Annual incidence<1 / 1 000 0000.02 / 100 000United StatesValue and classValidatedSEER Surveillance Epidemiology and end results [OTHER]_14999699[PMID]
Annual incidence<1 / 1 000 0000.009 / 100 000AustriaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.019 / 100 000BelgiumValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.029 / 100 000BulgariaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.029 / 100 000Czech RepublicValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.018 / 100 000EstoniaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.002 / 100 000FinlandValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.031 / 100 000FranceValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.019 / 100 000GermanyValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.043 / 100 000IcelandValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.022 / 100 000IrelandValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence1-9 / 1 000 0000.13 / 100 000ItalyValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.022 / 100 000LatviaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.003 / 100 000NorwayValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.005 / 100 000PolandValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.006 / 100 000PortugalValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.008 / 100 000SlovakiaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.009 / 100 000SpainValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.017 / 100 000SwitzerlandValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.022 / 100 000NetherlandsValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.016 / 100 000United KingdomValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.025 / 100 000EuropeValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

7

Sources for this card