Fibrolamellar Carcinoma (FLC)
Overview
FLC (Fibrolamellar Carcinoma) is a rare primary liver cancer arising predominantly in adolescents and young adults without underlying cirrhosis or hepatitis. It is histologically defined by polygonal tumor cells with abundant eosinophilic cytoplasm separated by lamellar fibrous stroma. The pathognomonic molecular event is a ~400 kb deletion on chromosome 19 that creates a DNAJB1-PRKACA chimeric fusion, leading to aberrant protein kinase A (PKA) catalytic subunit activity. FLC is a child of LIVER in OncoTree.
Cohorts in the corpus
- msk_impact_2017 — FLC was among 62 principal tumor types profiled in the MSK-IMPACT prospective pan-cancer sequencing initiative (n=10,945 tumors overall).
Recurrent alterations
- DNAJB1-PRKACA fusion — pathognomonic for fibrolamellar hepatocellular carcinoma; detected in the MSK-IMPACT pan-cancer cohort among the 1,875 rearrangements catalogued across 62 tumor types. PMID:28481359
- Pan-cancer fusion study (9,624 TCGA samples) confirmed DNAJB1–PRKACA as a liver-only fusion specific to fibrolamellar carcinoma (FLC), corroborating Dinh et al. 2017; FLC is a subtype of LIHC with a distinct and highly specific fusion driver PMID:29617662
Subtypes
- Fibrolamellar carcinoma is considered a distinct entity from conventional hepatocellular carcinoma (HCC); DNAJB1-PRKACA fusion is the defining molecular event and is absent in HCC.
Therapeutic landscape
- No targeted therapy directly approved for FLC at the time of the MSK-IMPACT publication; PRKACA-targeted strategies (PKA inhibitors) are under investigation. PMID:28481359
Sources
- PMID:28481359 — Zehir et al., MSK-IMPACT prospective pan-cancer sequencing (msk_impact_2017); DNAJB1-PRKACA identified as pathognomonic FLC rearrangement.
This page was processed by crosslinker on 2026-05-15. - PMID:29617662
This page was processed by wiki-cli on 2026-05-15.