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Article Abstract

Introduction: Immunoglobulin G4-related disease is a systemic fibroinflammatory disorder that affects the kidney, presenting as an immunoglobulin G4-related inflammatory pseudotumor. These renal inflammatory pseudotumors are usually solid, and cystic presentations have not been previously described.

Case Presentation: We report a 77-year-old man who presented with a Bosniak category III renal cystic mass and periaortic fibrous thickening. Increased serum immunoglobulin G4 levels indicated immunoglobulin G4-related disease; however, a malignant process could not be definitively ruled out. The patient underwent robot-assisted radical nephrectomy, and histopathologic assessment confirmed a cystic immunoglobulin G4-related inflammatory pseudotumor.

Conclusion: This is the first report of a renal immunoglobulin G4-related inflammatory pseudotumor with cystic features. Increased knowledge of immunoglobulin G4-related disease as a potential cause of cystic renal lesions, particularly in patients with systemic findings, may improve preoperative diagnosis.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC12408159PMC
http://dx.doi.org/10.1002/iju5.70074DOI Listing

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