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← Childhood Cancer Genomics (PDQ®)

HEALTH PROFESSIONAL · SOURCE READING

Molecular features of fibrolamellar carcinoma

Source: Childhood Cancer Genomics (PDQ®)–Health Professional Version, National Cancer Institute.

Source updated: April 30, 2025 · Captured 2026-09-09.

Selected source text with whitespace normalised. This Triangle page is not an NCI PDQ summary. Independent clinical review is pending.

Context: Liver Cancer / Fibrolamellar Carcinoma

Fibrolamellar carcinoma is a rare subtype of hepatocellular carcinoma observed in older children and young adults. It is characterized by an approximately 400 kB deletion on chromosome 19, which produces a chimeric transcript. This chimeric RNA codes for a protein containing the amino-terminal domain of DNAJB1, a homolog of the molecular chaperone DNAJ, fused in frame with PRKACA, the catalytic domain of protein kinase A.[14]

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For information about the treatment of childhood liver cancer, see Childhood Liver Cancer Treatment.

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Preserved source evidence · Independent clinical review pending · Not medical advice