Spontaneous Resolution of IgG4-Related Hepatic Inflammatory Pseudotumor Mimicking Malignancy

Abstract

Hepatic inflammatory pseudotumor (IPT) is characterized by a well-circumscribed benign tumor mimicking or often mistaken for a malignant lesion. A 48-year-old male presented to the hospital with complaints of epigastric pain, with initial laboratory findings showing mildly elevated alkaline phosphatase (140 U/L) with normal AST, ALT, bilirubin, and lipase, a CD4 count of 384, and an HIV viral load of > 10 million copies. The total IgG level was elevated to 2,228 mg/dL (normal IgG4 level 114 mg/dL). Contrast-enhanced MRI of the abdomen showed heterogeneous mass-like infiltration in the right lobe of the liver measuring 9.6 cm. The liver mass was biopsied which showed dense collagenous fibrosis with abundant lymphoplasmacytic infiltrates with 18 IgG4-positive plasma cells per high-power field. The patient was not given any treatment for this IPT. For more than 1 year of follow-up triple-phase CT scan of the liver was repeated, which showed no liver mass. As radiological images of hepatic IPTs, including IgG4-related hepatic IPT, mimic liver malignancy, histological analysis of the biopsy remains the cornerstone for the diagnosis. Symptomatic patients with IgG4-related hepatic IPT have shown improvement with corticosteroid use; however, spontaneous resolution has also been reported like in the present case.

Keywords: HIV-positive patient; IgG4-related diseases; Inflammatory pseudotumor; Lymphoplasmacytic infiltrate; Storiform fibrosis.

Fig. 1

a, b MRI of the abdomen showing a heterogenous, irregularly shaped mass infiltrating the right lobe of the liver, measuring 9.6 cm (yellow arrows).

Fig. 2

Histological images of hepatic biopsy stained by hematoxylin and eosin. a Abundant lymphoplasmacytic reaction with dense collagenous fibrosis. b Immunostaining showing IgG4 infiltrating the tissue and 18 positive plasma cells per high-power field.