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IgG4 Inflammatory Pseudotumor of the Kidney.

Alkhasawneh A, Alkhasawneh AN, Allan RW - Case Rep Urol (2012)

Bottom Line: Hyper-IgG4 disease is a rare systemic disorder that usually affects middle age males.Patients usually present with mass or masses in the involved organ that mimic neoplasia.We describe an unusual case of an IgG4-related pseudotumor of the kidney.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Immunology and Laboratory Medicine College of Medicine, University of Florida, P.O. Box 100275, Gainesville, FL 32610, USA.

ABSTRACT
Hyper-IgG4 disease is a rare systemic disorder that usually affects middle age males. It is characterized by elevated serum IgG4 levels and infiltration of organs by IgG4 positive plasma cells associated with fibrosis. Patients usually present with mass or masses in the involved organ that mimic neoplasia. While initially described in the pancreas, IgG4-related inflammatory tumors have been now described in many organs. We describe an unusual case of an IgG4-related pseudotumor of the kidney.

No MeSH data available.


Related in: MedlinePlus

(a) Gross examination from the partial nephrectomy specimen showing a relatively well circumscribed, firm, homogenous, white mass in the renal hilum (white bar = 1 cm), (b) low power showing a diffuse lymphoplasmacytic infiltrate associated with fibrosis that infiltrate between glomeruli, (c) medium power showing prominent fibrosis, and (d) IgG4 immunohistochemistry showing numerous IgG4+ plasma cells surrounding a glomerulus. No IgG4 positive plasma cells were identified in the glomerulus.
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fig1: (a) Gross examination from the partial nephrectomy specimen showing a relatively well circumscribed, firm, homogenous, white mass in the renal hilum (white bar = 1 cm), (b) low power showing a diffuse lymphoplasmacytic infiltrate associated with fibrosis that infiltrate between glomeruli, (c) medium power showing prominent fibrosis, and (d) IgG4 immunohistochemistry showing numerous IgG4+ plasma cells surrounding a glomerulus. No IgG4 positive plasma cells were identified in the glomerulus.

Mentions: Gross examination revealed a well-circumscribed 3.5 cm firm white homogenous mass that was confined to the kidney (Figure 1(a)). No hemorrhage or necroses were grossly identified. Microscopic examination revealed a well-circumscribed dense lymphoplasmacytic infiltrate with sclerotic bands within the tubulointerstitium of the renal cortex (Figures 1(b) and 1(c)). Immunohistochemical studies with IgG4 reveal diffuse reactivity within the plasma cells of the interstitium (mean of 75 cells/HPF per field, Figure 1(d)). No IgG4 positive plasma cells were present within the glomeruli. (Figure 1(d)). The morphologic and immunohistochemical findings were diagnostic of an IgG4-related pseudotumor. In light of this finding, the prior pancreatoduodenectomy slides were reviewed and were consistent with IgG4-related lymphoplasmacytic pancreatitis.


IgG4 Inflammatory Pseudotumor of the Kidney.

Alkhasawneh A, Alkhasawneh AN, Allan RW - Case Rep Urol (2012)

(a) Gross examination from the partial nephrectomy specimen showing a relatively well circumscribed, firm, homogenous, white mass in the renal hilum (white bar = 1 cm), (b) low power showing a diffuse lymphoplasmacytic infiltrate associated with fibrosis that infiltrate between glomeruli, (c) medium power showing prominent fibrosis, and (d) IgG4 immunohistochemistry showing numerous IgG4+ plasma cells surrounding a glomerulus. No IgG4 positive plasma cells were identified in the glomerulus.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3472528&req=5

fig1: (a) Gross examination from the partial nephrectomy specimen showing a relatively well circumscribed, firm, homogenous, white mass in the renal hilum (white bar = 1 cm), (b) low power showing a diffuse lymphoplasmacytic infiltrate associated with fibrosis that infiltrate between glomeruli, (c) medium power showing prominent fibrosis, and (d) IgG4 immunohistochemistry showing numerous IgG4+ plasma cells surrounding a glomerulus. No IgG4 positive plasma cells were identified in the glomerulus.
Mentions: Gross examination revealed a well-circumscribed 3.5 cm firm white homogenous mass that was confined to the kidney (Figure 1(a)). No hemorrhage or necroses were grossly identified. Microscopic examination revealed a well-circumscribed dense lymphoplasmacytic infiltrate with sclerotic bands within the tubulointerstitium of the renal cortex (Figures 1(b) and 1(c)). Immunohistochemical studies with IgG4 reveal diffuse reactivity within the plasma cells of the interstitium (mean of 75 cells/HPF per field, Figure 1(d)). No IgG4 positive plasma cells were present within the glomeruli. (Figure 1(d)). The morphologic and immunohistochemical findings were diagnostic of an IgG4-related pseudotumor. In light of this finding, the prior pancreatoduodenectomy slides were reviewed and were consistent with IgG4-related lymphoplasmacytic pancreatitis.

Bottom Line: Hyper-IgG4 disease is a rare systemic disorder that usually affects middle age males.Patients usually present with mass or masses in the involved organ that mimic neoplasia.We describe an unusual case of an IgG4-related pseudotumor of the kidney.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Immunology and Laboratory Medicine College of Medicine, University of Florida, P.O. Box 100275, Gainesville, FL 32610, USA.

ABSTRACT
Hyper-IgG4 disease is a rare systemic disorder that usually affects middle age males. It is characterized by elevated serum IgG4 levels and infiltration of organs by IgG4 positive plasma cells associated with fibrosis. Patients usually present with mass or masses in the involved organ that mimic neoplasia. While initially described in the pancreas, IgG4-related inflammatory tumors have been now described in many organs. We describe an unusual case of an IgG4-related pseudotumor of the kidney.

No MeSH data available.


Related in: MedlinePlus