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Pathology · Neoplasia: fibrolamellar carcinoma
Sample itemA 19-year-old woman has an abdominal mass. She has no cirrhosis or viral hepatitis, and serum AFP is normal. CT shows a large lobulated liver mass with a central scar. Histology shows large polygonal eosinophilic cells with prominent nucleoli lying between parallel bands of collagen. What is the diagnosis?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
C. Fibrolamellar hepatocellular carcinoma
Fibrolamellar carcinoma is a variant of hepatocellular carcinoma seen in adolescents and young adults without cirrhosis or hepatitis, typically with a normal AFP. It has oncocytic cells in lamellar fibrosis and a DNAJB1-PRKACA fusion, and the prognosis is better than for usual hepatocellular carcinoma if resectable. Focal nodular hyperplasia also has a central scar but is benign and shows no cytological atypia.
Option by option
A. Focal nodular hyperplasia
Focal nodular hyperplasia has bland hepatocytes with bile ductular proliferation in the scar and no atypia.
B. Hepatocellular adenoma
Hepatocellular adenoma is linked to oral contraceptives and bleeds, but lacks lamellar fibrosis and atypia.
C. Fibrolamellar hepatocellular carcinoma (correct)
Oncocytic atypical cells in lamellar fibrosis in a young non-cirrhotic liver define fibrolamellar carcinoma.
D. Hepatoblastoma
Hepatoblastoma occurs in infants and young children and has raised AFP and fetal or embryonal elements.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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