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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2021  |  Volume : 32  |  Issue : 5  |  Page : 1461-1464
A rare case of ureteric involvement in xanthogranulomatous pyelonephritis associated with giant ureteric calculus: An unusual entity

Department of Nephrology, Dayanand Medical College and Hospital, Ludhiana, Punjab, India

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Date of Web Publication4-May-2022


Xanthogranulomatous pyelonephritis (XGPN) is an uncommon chronic destructive granulomatous inflammation of the kidney with variable clinical and radiological presentation. Due to its similarities to other benign and malignant pathologies, a high index of suspicion is required for preoperative diagnosis, which will ensure appropriate management of this condition. The invasion into the surrounding structures such as pararenal spaces, psoas muscle, small bowel, diaphragm, lung or soft tissues has been reported. However, involvement of ureter is very rarely reported. We report a rare case of left-sided gross hydronephrosis with staghorn calculus with giant uretic calculi, postnephrectomy on the biopsy diagnosis of XGPN was made, which also revealed involvement of ureter also.

How to cite this article:
Sethi S, Mehta S, Gupta S. A rare case of ureteric involvement in xanthogranulomatous pyelonephritis associated with giant ureteric calculus: An unusual entity. Saudi J Kidney Dis Transpl 2021;32:1461-4

How to cite this URL:
Sethi S, Mehta S, Gupta S. A rare case of ureteric involvement in xanthogranulomatous pyelonephritis associated with giant ureteric calculus: An unusual entity. Saudi J Kidney Dis Transpl [serial online] 2021 [cited 2022 May 25];32:1461-4. Available from: https://www.sjkdt.org/text.asp?2021/32/5/1461/344768

   Introduction Top

Xanthogranulomatous pyelonephritis (XGPN) is an uncommon chronic destructive granulomatous inflammatory disease of the kidney accounting for 0.6% of all surgically proven cases of chronic pyelonephritis.[1],[2] XGPN is more commonly seen in middle-aged persons with female predominance.[2] Usually the patient presents with the history of recurrent episodes of fever, burning micturition, and flank pain, not responding to regular course of the antibiotics.[3] The unilateral presentation of this disease is more common. Its pathogenesis results from the sequalae of chronic and severe form of obstructing parenchymal inflammation. The inflammatory process starts in the pelvis, producing gradual destruction of medulla and cortex by direct extension focally, but gradually this progress to the diffuse involvement leading to the enlargement of the kidney. Hence, in late stages, renal pelvis is usually dilated and the surrounding parenchyma is converted to shaggy golden yellow tissue. It usually contains staghorn calculus in 75% of the patients.[4] The local extension of the inflammation into the surrounding perinephric space is common. Further, the invasion into the pararenal spaces, psoas muscle, small bowel, diaphragm, lung or soft tissues can also occur.[3] Involvement of ureter is very rare occurrence, with only rare cases have been reported literature.

   Case Report Top

A 52-year-old with no previous comorbidities presented with a history of low-grade fever, burning micturition, and left flank pain for the past one month. There was no history of any anorexia, vomiting, weight loss, or any other constitutional symptoms. There was neither history also of any pyuria, frothiness of urine, or gross hematuria nor history of any diabetes, hypertension, or any significant systemic illness in the past. The patient took oral antibiotics in past month for these complaints on outdoor basis without any significant improvement. On examination, he was moderately built with normal vitals. There was no significant finding on general physical examination. On per abdomen examination, there was swelling in the left flank region. His rest of systemic examination was unremarkable. His complete hemogram revealed leukocytosis (14500/dL) with neutrophilia (76%) and a mild microcytic hypochromic anemia, with a Hb of 10.2 g/ L (normal range 12–15 g/ L). His serum creatinine was 0.8 mg/dL with normal electrolytes and liver function tests. The initial urinalysis revealed pyuria with 70–80 pus cells per high power field. Erythrocyte sedimentation rate was also raised at 38 and quantitative C-reactive protein was elevated to 67 mg/ L. Urine culture grown  Escherichia More Details coli sensitive to meropenem and piperacillin. His X-ray of kidney, ureter, and bladder (KUB) showed left-sided staghorn calculus and there were two giant ureteric calculi occupying the whole of the ureter extending from pelvi-ureteric junction to the end of distal ureter [Figure 1]. Ultrasonography of the abdomen revealed gross left- sided hydroureteronephrosis with thinning of renal cortex with staghorn calculus with two giant ureteric calculi. These findings were confirmed by noncontrast computerized tomography scan of the KUB [Figure 2]. Percutaneous nephrostomy was done to drain the pus. The patient was treated with intravenous (IV) antibiotics and gradually the patient showed improvement. He became afebrile, dysuria, and pyuria improved. His scan was done, which revealed non-functioning left kidney with differential function of less than 5%. Hence, in view of large and grossly hydronephrotic infected non-functioning left kidney nephrectomy was panned. Left nephrectomy with ureterectomy was done, which revealed grossly hydronephrotic left kidney with only thin rim of renal cortex left. There was a staghorn calculus in the pelvis with two giant ureteric calculi. There was dilated, thickened, and fat containing ureter. The biopsy of the specimen revealed that the bulk of the remaining thin cortex was made up of fibroadipose tissue with involvement of the ureter. Histopathological examination [Figure 3]a and (Figure 3]b revealed diffuse XGPN with ureteric involvement.
Figure 1: X-ray of kidney, ureter, and bladder showing left-sided staghorn calculus and there are two giant ureteric calculi occupying the whole of the ureter.

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Figure 2: Noncontrast computerized tomography scan of the kidney, ureter, and bladder showing gross left-sided hydroureteronephrosis with thinning of renal cortex with staghorn calculus with two giant ureteric calculi.

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The authors obtained all appropriate consent forms from the patient for the publication of this case report.

   Discussion Top

XGPN is a rare type of chronic pyelonephritis associated with obstructive - lithiasis that, through an as of yet in completely elucidated etiology, results in a complete effacement of the normal renal parenchyma, with a severe decrease in the function of that renal unit.[5],[6] There is destruction of the renal parenchyma and replacement by solid sheets of lipid laden macrophages.[4] The process may be focal (10%–17%) or diffuse (83%–90%) with extension of inflammation outside the kidney. This entity is of particular interest, as the focal type may mimic renal cell carcinoma, while the diffuse form has imaging features which are characteristic.[7] This can be classified in three stages: stage I (nephric XGP) in which the process is limited to the kidney; stage II (perinephric XGP) where the inflammation has extended to the perirenal fat, and stage III (paranephric XGP) where the inflammation extends beyond the kidney and can invade neighboring structures, such as the psoas muscle, the pancreas, the spleen, and the duodenum.[5],[8],[9]

In imaging, computed tomography (CT) is the modality of choice, though plain radiograph and ultrasound (US) are the initial examinations.[7] Plain scout radiograph shows a staghorn calculus. US typically shows an enlarged kidney with multiple hypoechoic anechoic areas with a central staghorn calculus. The renal parenchyma is thinned and corticomedullary differentiation is lost. XGPN has mainly to be differentiated from renal replacement lipomatosis. Both show a staghorn calculus with a nonfunctioning kidney. However, on CT, there is low attenuation material (+15 to -15 HU) filling the calyces in XGPN, whereas renal replacement lipomatosis shows attenuation of pure fat.[10] Other fat containing tumors such as angiomyolipoma, lipoma, or liposarcoma may be considered in differential diagnosis, but the absence of a staghorn calculus and a normally functioning kidney differentiates it from XGPN.[10] CT is also the modality of choice in depicting perinephric extension.[4]

Surgery remains the main modality of the treatment for XGP, while also providing a definitive diagnosis although there are case reports of successful treatment by only medical therapy.[5] Sometimes, percutaneous drainage of collecting system may be necessary to evacuate a collection or abscess, before surgery, as was the case with our patient. A nephroureterectomy was performed and there was an extension of the inflammatory process beyond the kidney to ureter in our case. Diagnosis of XGPN was made postnephrectomy on biopsy in our case, which also revealed involvement of ureter also. There were two giant ureteric stones involving the whole of the ureteric length which is not even reported in the literature. The involvement of ureter by XGPN is very unusual entity, which is rarely reported in the literature.

In conclusion, XGPN is a rare entity, sometimes diagnosis of this made postnephrectomy on the biopsy, as in our case, only thin portion of renal parenchyma was left because of underlying gross hydronephrosis. Involvement of ureter is very rare entity and even so such giant ureteric stones were not reported in the literature.

Conflict of interest: None

   References Top

Malek RS, Greene LF, DeWeerd JH, Farrow GM. Xanthogranulomatous pyelonephritis. Br J Urol 1972;44:296-308.  Back to cited text no. 1
Nandedkar SS, Malukani K, Sakhi P. Xanthogranulomatous pyelonephritis masquerading as a tumor in an infant. Indian J Urol 2014;30: 354-6.  Back to cited text no. 2
[PUBMED]  [Full text]  
Davidson AJ, Hartman DS. Diagnostic set: Large multifocal, unilateral. In: Radiology of the Kidney & Urinary Tract. 2nd ed. WB Sunders; 1994. p. 309-13.  Back to cited text no. 3
Goldman SM, Hartman DS, Fishman EK, Finizio JP, Gatewood OM, Siegelman SS. CT of xanthogranulomatous pyelonephritis: Radiologic-pathologic correlation. AJR Am J Roentgenol 1984;142:963-9.  Back to cited text no. 4
Iumanne S, Shoo A, Akoko L, Scanlan P. Case report: Xanthogranulomutous pyelonephritis presenting as “Wilms’ tumor”. BMC Urol 2016;16:36.  Back to cited text no. 5
Tüysüz G, Tayfun F, Canpolat F, et al. A case of xanthogranulomatous pyelonephritis mimicking Wilms tumor. Turk J Pediatr 2015; 57:409-12.  Back to cited text no. 6
Kenney PJ, Breatnach ES. Chronic inflammation. In: Pollock HM, editor. Clinical Urography an Atlas and Text book of Urological Imaging. WB Sunders; 1990. p. 807-35.  Back to cited text no. 7
Hendrickson RJ, Lutfiyya WL, Karrer FM, Furness PD 3rd, Mengshol S, Bensard DD. Xanthogranulomatous pyelonephritis. J Pediatr Surg 2006;41:e15-7.  Back to cited text no. 8
Ubetagoyena M, Fernandez Martin M, Gondra Sangroniz L. Xantogranulomatous pyelonephritis in children. Arch Esp Urol 2014;67: 214-7.  Back to cited text no. 9
Frederick MG, Hall BP. Genitourinary case of the day. Replacement lipomatosis of the kidney. AJR Am J Roentgenol 1995;165:200.  Back to cited text no. 10

Correspondence Address:
Sudhir Mehta
Department of Nephrology, Dayanand Medical College and Hospital, Ludhiana, Punjab
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1319-2442.344768

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  [Figure 1], [Figure 2], [Figure 3]


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