Hepatic inflammatory pseudotumor (IPT) is a rare, benign, tumor-like lesion of the liver characterized by a proliferation of fibrous connective tissue and infiltration of inflammatory cells, including plasma cells, lymphocytes, and macrophages. Despite its non-neoplastic nature, IPT often mimics malignant hepatic tumors such as intrahepatic cholangiocarcinoma, posing significant diagnostic challenges. The etiology remains unclear, though proposed mechanisms include autoimmune responses, infectious triggers, and IgG4-related disease. Histologically, IPTs are classified into two subtypes: fibrohistiocytic and lymphoplasmacytic. The fibrohistiocytic type typically presents in the peripheral hepatic parenchyma and features xanthogranulomatous inflammation and multinucleated giant cells. The lymphoplasmacytic type, often associated with IgG4-related disease, is found near the hepatic hilum and exhibits storiform fibrosis, obliterative phlebitis, and abundant IgG4-positive plasma cells. Radiologically, IPTs display variable imaging features, including hypoattenuating masses on CT and hyperintense lesions on T2-weighted magnetic resonance imaging (MRI). Enhancement patterns differ between subtypes, with delayed hypoenhancement and duct-penetrating signs more common in IgG4-related IPTs. Due to their nonspecific appearance, IPTs are frequently misdiagnosed as abscesses or malignancies, leading to unnecessary surgical interventions. Diagnosis relies on a combination of imaging, histopathology, and immunohistochemistry. While some IPTs regress spontaneously or respond to corticosteroids, others require surgical resection for definitive diagnosis and symptom relief. Improved recognition of imaging and histologic patterns is essential to avoid overtreatment and guide appropriate management.

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Hepatic Inflammatory Pseudotumor

  • Takeshi Takamoto,
  • Daisuke Ban

摘要

Hepatic inflammatory pseudotumor (IPT) is a rare, benign, tumor-like lesion of the liver characterized by a proliferation of fibrous connective tissue and infiltration of inflammatory cells, including plasma cells, lymphocytes, and macrophages. Despite its non-neoplastic nature, IPT often mimics malignant hepatic tumors such as intrahepatic cholangiocarcinoma, posing significant diagnostic challenges. The etiology remains unclear, though proposed mechanisms include autoimmune responses, infectious triggers, and IgG4-related disease. Histologically, IPTs are classified into two subtypes: fibrohistiocytic and lymphoplasmacytic. The fibrohistiocytic type typically presents in the peripheral hepatic parenchyma and features xanthogranulomatous inflammation and multinucleated giant cells. The lymphoplasmacytic type, often associated with IgG4-related disease, is found near the hepatic hilum and exhibits storiform fibrosis, obliterative phlebitis, and abundant IgG4-positive plasma cells. Radiologically, IPTs display variable imaging features, including hypoattenuating masses on CT and hyperintense lesions on T2-weighted magnetic resonance imaging (MRI). Enhancement patterns differ between subtypes, with delayed hypoenhancement and duct-penetrating signs more common in IgG4-related IPTs. Due to their nonspecific appearance, IPTs are frequently misdiagnosed as abscesses or malignancies, leading to unnecessary surgical interventions. Diagnosis relies on a combination of imaging, histopathology, and immunohistochemistry. While some IPTs regress spontaneously or respond to corticosteroids, others require surgical resection for definitive diagnosis and symptom relief. Improved recognition of imaging and histologic patterns is essential to avoid overtreatment and guide appropriate management.