PMC4899571_01
71-year-old male with IgG4-related sclerosing disease. (A) Axial precontrast CT image demonstrates a predominantly hypoattenuated, ill-defined mass of the right renal hilum and right renal pelvis. (B) Postcontrast image during the corticomedullary phase demonstrates minimal to no enhancement of the mass. (C-D) Delayed images demonstrate the mass surrounding (but not significantly distorting) the collecting system with minimal hydronephrosis.
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CT of the abdomen demonstrated a heterogeneous, low-attenuating, right renal pelvis soft-tissue mass with indistinct borders and no significant enhancement (Fig. 1).
71-year-old male with IgG4-related sclerosing disease. Gross specimen of the right kidney demonstrates an enlarged kidney with soft-tissue infiltration of the hilum and perinephric adipose tissue.
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The surgical pathology from the right nephrectomy demonstrated a fibroinflammatory process with acute and chronic inflammatory infiltrate, lymphoid aggregates, plasma cells, macrophages, and rare multinucleated giant cells (Figure 2, Figure 3).
71-year-old male with IgG4-related sclerosing disease. (A) Low-power H&E stain shows diffuse infiltration of the renal interstitium by eosinophils, plasma cells, and lymphocytes. (B) High-power H&E stain shows eosinophils, lymphocytes, and plasma cells. (C) H&E stain of perinephric adipose tissue shows the same infiltrate of eosinophils, plasma cells, and lymphocytes.
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The surgical pathology from the right nephrectomy demonstrated a fibroinflammatory process with acute and chronic inflammatory infiltrate, lymphoid aggregates, plasma cells, macrophages, and rare multinucleated giant cells (Figure 2, Figure 3).
71-year-old male with IgG4-related sclerosing disease. Immunochemical stains show plasma cells with (A) IgG kappa (B) IgG lambda, and (C) an excess number of IgG4-positive stains compatible with IgG4-related sclerosing disease.
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CD3, CD4, and CD20 highlighted B and T lymphocytes (Fig. 4).