Spontaneous regression of hepatic inflammatory pseudotumor with primary biliary cirrhosis: case report and literature review

Abstract

Hepatic inflammatory pseudotumor (IPT) is a rare benign non-neoplastic lesion characterized by proliferating fibrous tissue infiltrated by inflammatory cells. The exact etiology of IPT remains unclear. Although the association of IPT with systemic inflammatory disorders has been well established, a specific relationship with cholangitis is distinctly rare. We report a case of spontaneous regression of hepatic IPT with primary biliary cirrhosis (PBC). To date, only two cases of IPT with PBC have been reported. In our case, however, IPT developed during the course of improvement of cholangitis of PBC induced by effective treatment, differing from two previously reported cases. Our case indicates that the development of IPT does not also relate to the activity of cholangitis and/or hyper gamma-globulinemia, since our case was confirmed radiologically to be free of IPT when biliary enzymes and immunoglobulins were much higher than the corresponding values on admission. Comparison of our case with the two previously reported cases suggests that IPT occurring with PBC does not represent the same disease entity or be a bystander for PBC.

Figure 1

A, B: Whole body F-18 fluorodeoxyglucose (FDG) positron emission tomography (PET) scan showed a 3-cm spherical mass in the right lobe of the liver. A:Coronal sectional view, B: horizontal sectional view. C: Enhanced CT showed a 3-cm spherical low-density mass (arrow) in the right lobe of the liver. D: Another enhanced CT performed 3 mo after targeted liver biopsy showed complete resolution of the mass.

Figure 2

A: Liver biopsy specimen from the non-tumorous liver. Histopathological findings are consistent with primary biliary cirrhosis. The enlarged portal tract with damaged bile ducts in florid lesion of non-suppurative destructive cholangitis is infiltrated by inflammatory mononuclear cells. H&E, X100. B: Liver biopsy specimen shows clear-cut boundary between the “tumor” and liver parenchyma. Azan-Mallory, ×100. C:The “tumor” is composed of chronic inflammatory cells including lymphocytes and plasma cells. H&E, × 400. D: The “tumor” is composed of fibrous tissue, thick hyalinized collagen bundles with disappearance of liver parenchyma. H&E, ×400.