Picture159 yo F w/ insidious onset of jaundice, ascites, & elevated transaminases. Prior liver biopsies revealed portal fibrosis & cholestasis. Patient deemed to have cryptogenic cirrhosis & transplantation performed.

 What is the most likely diagnosis?

A. Portal fibrosis and cirrhosis.
B. Hepatoportal sclerosis.
C. Hepatic amyloidosis.
D. Thorotrast deposition.



Answer

Picture3The answer is “C”, hepatic amyloidosis.
Sections show liver parenchyma with expansion of portal tracts containing little to no inflammation, cholestasis, and bridging fibrosis. The expanded portal tracts appear acellular, containing amorphous material. Trichrome stain (not pictured) highlights bridging fibrosis, with only faint staining within the portal tracts. The Congo red stain highlights the amorphous material in the portal tracts, with apple-green birefringence on polarized light (inlet), confirming the presence of amyloid.
Amyloidosis is usually seen in the systemic form, but 10-20% of cases are localized within the liver. Amyloid deposition most frequently occurs within sinusoidal spaces but can be seen in the connective tissue of the portal tracts. Unfortunately, the pattern of amyloid deposition is not a reliable method of distinguishing primary vs secondary amyloidosis.

Odze, Robert D. “Vascular Disorders of the Liver.” Odze and Goldblum Surgical Pathology of the GI Tract, Liver, Biliary Tract and Pancreas, Third Edition, Saunders, 2015, pp. 1396–1397.

 Contributed by Chrag Patel, M.D.