Hepatic involvement in systemic amyloidosis is relatively common at autopsy but is rarely the dominant clinical presentation, and its cross-sectional imaging features are often nonspecific. We report a case of biopsy-proven systemic amyloidosis in a 55-year-old woman in whom radiologic hepatic abnormalities were identified more than one year before the development of overt renal dysfunction, and in whom a heterogeneous parenchymal enhancement pattern on cross-sectional imaging was initially interpreted as a primary vascular disorder of the liver with perfusion abnormality, with hereditary hemorrhagic telangiectasia (HHT) and portosinusoidal vascular disease (PSVD) considered the leading differential diagnoses. Dynamic contrast-enhanced CT and gadoxetic acid–enhanced MRI showed heterogeneous arterial-phase parenchymal enhancement with hepatic arterial hypertrophy and a relatively small left portal vein, but no surface nodularity, splenomegaly, or other findings of portal hypertension. Percutaneous biopsy of the right hepatic lobe demonstrated portal lymphocytic infiltration with marked eosinophils and irregular sinusoidal dilatation, without histologic features of cirrhosis. Approximately one year later, the patient developed progressive renal dysfunction, and renal biopsy revealed extensive glomerular and arterial amyloid deposition with apple-green birefringence on Congo red staining under polarized light. Retrospective re-evaluation of the prior liver biopsy with Congo red staining demonstrated amyloid deposition confined to the walls of blood vessels. The systemic workup favored AA (secondary) amyloidosis. This case illustrates that hepatic amyloidosis with vessel-wall–predominant deposition may mimic primary vascular disorders of the liver on cross-sectional imaging and should be considered when heterogeneous parenchymal perfusion is encountered in the absence of definite cirrhosis.
Park et al. (Tue,) studied this question.