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CASE REPORT
Abstract
Xanthogranulomatous pyelonephritis is an uncommon chronic destructive disease process of renal parenchyma, associated with
recurrent urinary tract infection. It is seen predominantly in females with no age specificity. The most common symptoms are
flank or abdominal pain, fever, palpable mass, and gross hematuria. The common laboratory findings are leukocytosis and ane-
mia. Urine cultures most often reveal Escherichia coli and Proteus mirabilis. Computed tomography is the mainstay of diagnostic
imaging for xanthogranulomatous pyelonephritis. Histologically, xanthogranulomatous pyelonephritis presents a granulomatous
inflammatory infiltrate mainly composed of lymphocytes, plasma cells, foamy histiocytes, and multinucleated giant cells. The
differential diagnosis includes clear cell renal cell carcinoma, sarcomatoid renal cell carcinoma, leiomyosarcoma, malakoplakia,
tuberculosis, and interstitial nephritis. Treatment includes antibiotics and surgery. In this article, we report a case of xanthogran-
ulomatous pyelonephritis in a 38-year-old male patient with recurrent urinary tract infection.
Keywords: differential diagnosis; interstitial nephritis; leiomyosarcoma; lipid-ladden foamy macrophages; malakoplakia; recurrent Urinary tract
infection; Xanthogranulomatous pyelonephritis
Received: 25 May 2015; Accepted after revision: 20 August 2017; Published: 29 September 2017.
Author for correspondence: Kafil Akhtar, Department of Pathology, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh,
UP, India. Email: [email protected]
How to cite: Akhtar K et al. Xanthogranulomatous pyelonephritis: A rare presentation, J Ren Hepat Disord 2017;1(2):52–56.
DOI: http://dx.doi.org/10.15586/jrenhep.2017.19
License: This open access article is licensed under Creative Commons Attribution 4.0 International (CC BY 4.0).
http://creativecommons.org/licenses/by/4.0
Introduction frequently than men, with male to female ratio of 3:7 (2, 4).
Xanthogranulomatous pyelonephritis (XGP) is a chronic de- XGP is characterized by an infectious phlegmon arising in
structive granulomatous inflammation of renal parenchyma, the renal parenchyma, in immunocompromised person with
first described by Schlagenhaufer in 1916 (1, 2). There are associated urinary tract infection and/or urolithiasis. The
three recognized patterns of the disease: diffuse, segmental, clinical presentation is nonspecific, which leads to difficulty
and focal. Segmental XGP is characterized by segmental in diagnosis with other kidney diseases. Computed tomog-
involvement, focal disease is mainly cortical, while diffuse raphy (CT) is a reliable and accurate imaging modality in
XGP largely involves the renal parenchyma (3). XGP is an characterizing its extrarenal involvement, thereby leading
uncommon entity, accounting for about 0.6% of histologi-
to its diagnosis. The treatment is surgical and consists of
cally documented cases of chronic pyelonephritis (4, 5).
nephrectomy. We report a case of XGP in a 38-year-old
The mean age of occurrence of XGP is 45.2 years,
male patient with recurrent urinary tract infection.
with age range of 2–84 years (2, 4). Women are affected more
Discussion
XGP is a serious, chronic inflammation of the renal par-
enchyma associated with indolent bacterial infection. The
disease process begins in the renal pelvis and extends into
the medulla and cortex which are gradually destroyed
and replaced by lipid-laden macrophages or xanthoma
cells (1). XGP is usually seen in the fifth and sixth de-
cades of life, with a female preponderance. Most cases
of XGP are unilateral; however, bilateral disease has also
been reported and is generally fatal (2). The exact etiol-
ogy of XGP is unknown, but it is usually associated with
long-term renal obstruction and subsequent infection
(3). Calculi may be seen in 75–86% of the patients, but
our patient did not have any renal calculus (4). Culture
shows Proteus and E. coli infection in 30–40% of cases
(5). Additional predisposing factors include ureteropel-
vic junction syndrome, bladder tumor, and chronic inter-
stitial nephritis. Comorbid conditions include pregnancy,
diabetes mellitus, rheumatoid arthritis, chronic viral Figure 1. Gross specimen of kidney revealing a large firm
hepatitis C, cirrhosis, and obesity (6). Patients usually yellow mass with multiple foci of necrosis.
A B
Figure 2. (A) Histopathology showing focal areas of lipid-laden “foamy” macrophages accompanied by neutrophils, lympho-
cytes, and plasma cells (hematoxylin and eosin, 100x). (B) High power of Figure A (400x).
Microscopically, XGP is characterized by a diffuse or focal and epithelial membrane antigen. Leiomyosarcoma, another
granulomatous mixed inflammatory infiltrate with fibrosis spindle cell lesion, is also in the differential diagnosis; it
and cholesterol clefts in the background. The inflammatory demonstrates interlacing bundles of spindle cells with blunt-
infiltrate is composed of a variable number of xanthomatous ended nuclei and eosinophilic cytoplasm. These cells are pos-
histiocytes with foamy cytoplasm, neutrophils, lymphocytes, itive for desmin and smooth muscle actin (8).
plasma cells, and multinucleated giant cells. Variable degree Two benign closely related entities are malakoplakia and
of renal tubular atrophy, tubular dilatation and microab- megalocytic interstitial nephritis. The key characteristic of
scesses with spindle cell proliferation can be observed (11). both lesions is the periodic acid–Schiff diastase-positive ma-
The lesion shows diffuse positivity for CD68 and vimentin, terial in the cytoplasm of the histiocytes. Malakoplakia of
and negativity for smooth muscle actin, desmin, and epithe- the kidney is primarily a disease of the renal pelvis with in-
lial markers (6). The xanthomatous cells and macrophages volvement of the renal parenchyma and Michaelis–Gutmann
show positive cytoplasmic staining for a1-antritrypsin and bodies are characteristic of the lesion (9). Other differential
lysozyme (11). diagnoses include tuberculosis and renal abscess. Character-
The differential diagnosis of XGP includes clear cell RCC, istic histology and special and immunohistochemistry stains
sarcomatoid RCC, leiomyosarcoma, malakoplakia, tubercu- usually lead to the right diagnosis.
losis, and interstitial nephritis (Table 1). The lipid-laden xan- Medical therapy alone is inadequate to treat XGP; an-
thomatous cells in XGP may mimic the clear cells of clear tibiotics are a temporary measure for patients requiring
cell RCC. The xanthomatous cells have a foamy c ytoplasm medical work-up prior to nephrectomy. Total nephrec-
compared with the clearer cytoplasm of clear cells. Adequate tomy is the gold standard of treatment for XGP, unless
sampling should reveal granulomatous inflammation of XGP both sides are affected in which case partial nephrectomy
and the papillary structures of papillary RCC. Immunohis- is performed (9). The prognosis is considered to be good
tochemical study can play an essential role in the differen- after treatment (10).
tial diagnosis. XGP is diffusely positive for CD68. RCC is
usually positive for CD10 and epithelial membrane antigen. Conclusion
Vimentin can stain positive for Vimentin can stain positive
XGP is an uncommon encounter on the surgical pathology
for both XGP and RCC. XGP with prominent spindle cell
bench and is associated with long-term urinary tract obstruc-
proliferation may mimic sarcomatoid RCC; the differenti- tion and infection. It mimics various benign and malignant
ation relies on demonstration of markedly atypical spindle conditions, both clinically and pathologically. It requires a
cells and coexisting epithelial cell components. Sarcomatoid combination of clinical presentation, imaging studies, and
RCC demonstrates at least focal positivity for cytokeratin biopsies for a definite diagnosis.
CK, cytokeratin; EMA, epithelial membrane antigen; AFB, acid fast bacilli; ZN, ziehl neelsen; PAS, periodic acid schiff; SMA, smooth
muscle actin; IHC, immunohistochemistry; RCC, renal cell carcinoma; TB, tuberculosis; USG, ultrasound; CT scan, computed tomography
scan; SMA; XGP, xanthogranulomatous pyelonephritis.
3. Loffroy R, Guiu B, Watfa J, Michel F, Cercueil JP, Krause D. correlative histology. Cytopathology 2009;20:50–5. https://doi.
Xanthogranulomatous pyelonephritis in adults: Clinical and ra- org/10.1111/j.1365-2303.2007.00515.x
diological findings in diffuse and focal forms. Arch Pathol Lab 8. Ho CI, Wen YK, Chen ML. Xanthogranulomatous pyelone-
Med 2011;135:671–4. phritis successfully treated with antibiotics only. J Chin Med
4. Zugor V, Schott GE, Labanaris AP. Xanthogranulomatous py- Assoc 2008;71(12):643–5. http://dx.doi.org/10.1016/S1726-4901
elonephritis in childhood: A critical analysis of 10 cases and (09)70008-5
of the literature. Urology 2007;70(1):157–60. http://dx.doi. 9. Tsai KH, Lai MY, Shen SH, Yang AH, Su NW, Ng YY. Bilateral
org/10.1016/j.urology.2007.02.068 xanthogranulomatous pyelonephritis. J Chin Med Assoc 2008;71(6):
5. Su YJ, Lai YC, Chou CY, Chang WH. Ischemic colitis second- 310–14. http://dx.doi.org/10.1016/S1726-4901(08)70128-X
ary to xanthogranulomatous pyelonephritis. Int J Infect Dis 10. Hitti W, Drachenberg C, Cooper M. Xanthogranulomatous
2009;13(3):89–91. https://doi.org/10.1016/j.ijid.2008.06.033 pyelonephritis in a renal allograft associated with xanthogranu-
6. Wen YK, Chen ML. Xanthogranulomatous pyelonephritis lomatous diverticulitis: Report of the first case and review of the
complicated by emphysematous pyelonephritis in a hemodi- literature. Nephrol Dial Transplant 2007;22(11):3344–7. http://
alysis patient. Clin Nephrol 2007;68(6):422–7. http://dx.doi. dx.doi.org/10.1093/ndt/gfm458
org/10.5414/CNP68422 11. Lya Duarte R, Marinus ML, Mauricio C, Geraldo BS, Eliza-
7. Masoom S, Venkataraman G, Jensen J, Flanigan RC, Wojcik beth DF, Daher D. Emphysematous and xanthogranulomatous
EM. Renal FNA-based typing of renal masses remains a use- pyelonephritis. Braz J Infect Dis 2010;14(4):374–6. http://dx.
ful adjunctive modality: Evaluation of 31 renal masses with doi.org/10.1016/S1413-8670(10)70078-7