Abstract

IntroductionInflammatory pseudotumor is rare benign mass composed of chronic inflammatory cell infiltration and proliferating fibrous tissue. Some cases of inflammatory pseudotumor show abundant infiltrating IgG4-positive plasma cells and obliterative phlebitis, which are the pathologic hallmarks of autoimmune pancreatitis.Case presentationA 77-year-old Japanese man was admitted to our hospital because of epigastric pain. A solitary mass with delayed enhancement by dynamic computed tomography was present in the left hepatic lobe. Endoscopic retrograde cholangiography showed only segmental stenosis of the left intrahepatic bile duct. No abnormal findings were detected in the pancreas. The patient was clinically diagnosed as having intrahepatic cholangiocarcinoma and underwent surgery. Histological examination of the hepatic mass and bile duct wall showed abundant IgG4-positive plasma cell infiltration with obliterative phlebitis. The final diagnosis was IgG4-related hepatic inflammatory pseudotumor with sclerosing cholangitis. Delayed enhancement by computed tomography is a characteristic feature of IgG4-related inflammatory pseudotumor similar to that of autoimmune pancreatitis.ConclusionIgG4-related hepatic inflammatory pseudotumor unassociated with autoimmune pancreatitis should be one of the entities considered for differential diagnosis of liver tumors. Delayed enhancement on computed tomography might be useful finding for diagnosing IgG4-related hepatic inflammatory pseudotumor.

Highlights

  • Inflammatory pseudotumor is rare benign mass composed of chronic inflammatory cell infiltration and proliferating fibrous tissue

  • Some cases of inflammatory pseudotumor show abundant infiltrating IgG4-positive plasma cells and obliterative phlebitis, which are the pathologic hallmarks of autoimmune pancreatitis

  • IgG4-related hepatic inflammatory pseudotumor unassociated with autoimmune pancreatitis should be one of the entities considered for differential diagnosis of liver tumors

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Summary

Introduction

Inflammatory pseudotumor (IPT) of the liver is a rare, benign lesion composed of chronic inflammatory cell infiltration and proliferating fibrous tissue This disease is becoming an important entity to consider for differential diagnosis in patients with hepatic space-occupying lesions. Some cases of IPT show infiltration of abundant IgG4-positive plasma cells and obliterative phlebitis, which are the pathologic hallmarks of AIP [7,8]. Magnetic resonance cholangiopancreatography (MRCP) revealed stricture of the intrahepatic bile ducts with prestenotic dilatation in the left lateral segment (Figure 2). Macroscopic examination of the resected left hepatic lobe revealed a whitish nodular mass measuring 4 x 3 cm spreading along the hilar bile duct to B3 (Figure 3A). IPTs of the plasma cell granuloma type show infiltration of numerous IgG4-positive plasma cells and obliterative phlebitis, which are the pathologic hallmarks of AIP [7,8]. The final diagnosis was IgG4-related hepatic IPT with SC

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