Hepatocellular carcinoma (HCC) arising in non-cirrhotic liver most characteristically occurs in which specific subtype?

• A HBV-associated HCC in endemic areas
• B Fibrolamellar HCC — arises in young adults in non-cirrhotic liver with DNAJB1-PRKACA fusion ✓
• C Alcohol-related HCC in advanced steatohepatitis
• D NASH-associated HCC in metabolic syndrome

Explanation

Fibrolamellar HCC (FLHCC) is a distinct clinicopathological entity arising in young adults (teens to 30s) in non-cirrhotic, otherwise normal liver, often with elevated AFP (mild) but characteristically with elevated PIVKA-II. Its molecular hallmark is the DNAJB1-PRKACA fusion gene resulting from a ~400 kb deletion on chromosome 19q, leading to aberrant PRKACA (catalytic subunit of PKA) overexpression. Microscopically, large polygonal tumor cells with prominent nucleoli are separated by abundant fibrous lamellae. This differentiates it from conventional HCC which typically requires cirrhosis.

Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.