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Kidney non-tumor

Tubular and interstitial diseases

Xanthogranulomatous pyelonephritis

Reviewers: Nikhil Sangle, M.D. (see Reviewers page)
Revised: 21 August 2014, last major update August 2012
Copyright: (c) 2003-2014, PathologyOutlines.com, Inc.


● Rare, severe, atypical form of chronic pyelonephritis due to infection (E. coli, Proteus) or stones, but resembling renal cell carcinoma; correct preoperative diagnosis is unusual (Arch Pathol Lab Med 2011;135:671, Arch Dis Child 1999;81:483)
● Characterized by foamy histiocytes replacing renal parenchyma, due to
● High index of suspicion necessary for preoperative diagnosis (Int Urol Nephrol 2011;43:15), intraoperative urine culture may be helpful (Tunis Med 2010;88:427)

Clinical features

● Destructive inflammatory process that may complicate chronic pyelonephritis
● Usually unilateral, ages 40+, 2/3 women
● Associated with pelvicalyceal obstruction and ulceration
● Rarely coexists with squamous cell carcinoma (J Cancer Res Ther 2010;6:339), renal cell carcinoma (ScientificWorldJournal 2009;9:5)

Case reports

● 51 year old woman with Liesegang rings (Patholog Res Int 2010;2010:602523
● 68 year old man with cirrhosis and 2.9 cm renal mass (Case of the Week #323)
● 68 year old woman with fever of unknown origin and renal mass (Heart Lung 2012 May 31 [Epub ahead of print])

Gross description

● Multiple yellow nodules around calyces, may form a mass and be infiltrative

Gross images

Various images

Micro description

● Replacement of renal parenchyma with CD68+ foamy histiocytes, occasional multinucleated giant cells and inflammatory cells

Micro images

Various images

Liesegang rings

Case of the Week #323:

Various images


Differential diagnosis

Malakoplakia: Michaelis-Gutmann bodies
Renal clear cell carcinoma: cells with clear cytoplasm may resemble histiocytes, but are keratin+, CD68-; arranged in compact, tubulocystic, alveolar or rarely papillary patterns; often glassy hyaline globules; usually nuclear grade 2 or higher; chicken wire / delicate vasculature is common (sinusoids near each packet of cells)
Renal replacement lipomatosis: atrophic renal parenchyma is replaced by fatty tissue, not xanthoma cells (Rev Assoc Med Bras 2011;57:262)

End of Kidney non-tumor > Tubular and interstitial diseases > Xanthogranulomatous pyelonephritis

Ref Updated: 8/21/12

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