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Urological Science 26 (2015) 69e71

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Urological Science

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Case report

Staghorn calculi and xanthogranulomatous pyelonephritis associatedwith transitional cell carcinoma

Chao-Wei Tseng a, Wei-Nung Jim Chen a, Guang-Dar Juang a,Thomas I-Sheng Hwang a, b, c, *

a Division of Urology, Department of Surgery, Shin-Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwanb Division of Urology, School of Medicine, Fu-Jen Catholic University, New Taipei, Taiwanc Department of Urology, Taipei Medical University, Taipei, Taiwan

a r t i c l e i n f o

Article history:Received 4 August 2014Received in revised form11 December 2014Accepted 14 December 2014Available online 4 February 2015

Keywords:metastasisrenal pelvisstaghorn calculustransitional cell carcinomaxanthogranulomatous pyelonephritis

* Corresponding author. Division of Urology, DeparWu Ho-Su Memorial Hospital, No. 95, Wen Chang RCity, Taiwan.

E-mail address: M001009@ms.skh.org.tw (T.I.-S. H

http://dx.doi.org/10.1016/j.urols.2014.12.0061879-5226/Copyright © 2015, Taiwan Urological Asso

a b s t r a c t

Untreated staghorn calculi can cause xanthogranulomatous pyelonephritis (XGP), diminished renalfunction, and renal malignancy. Squamous cell carcinoma (SCC) of the upper urinary tract is associatedwith kidney stones and chronic infection, but their association with transitional cell carcinoma (TCC) hasnot been proven and has rarely been reported in literature. We present a rare case of staghorn calculi andXGP associated with TCC.Copyright © 2015, Taiwan Urological Association. Published by Elsevier Taiwan LLC.Open access under CC BY-NC-ND license.

1. Introduction

Staghorn calculi are large and branched stones that can fill anentire renal calyceal system. Persistent chronic irritation and/orinfection by the staghorn calculi can cause xanthogranulomatouspyelonephritis (XGP), diminished renal function, or renal malig-nancy.1,2 The most common type of cancer associated with stag-horn calculi is squamous cell carcinoma (SCC), with a varyingincidence of 18e100%,3,4 followed by the rarely reported transi-tional cell carcinoma (TCC). TCC of the upper urinary tract is uni-formly fatal unless it is treated appropriately. In a multicenter studyof 10,355 patients with TCC, the 5-year cancer-specific survival ratewas approximately 54%.5 TCC has a recurrence rate of 50% in thebladder6 and a high metastases rate up to 75% in the lymph nodes.7

Thus, early and proper management of TCC is essential for patientsurvival.

Here, we report an extremely rare case of a patient who hadstaghorn calculi and XGP associated with TCC.

tment of Surgery, Shin-Kongoad, Shih Lin District, Taipei

wang).

ciation. Published by Elsevier Taiw

2. Case Report

A 75-year-old female presented with dysuria, high urinationfrequency, and right flank pain for 5 days. She had a 4-year historyof medically controlled hypertension and Stage 3 chronic kidneydisease as a comorbid condition. Her family history and physicalexamination were noncontributory. She had not undergone anysurgical procedures the previous day. The urine analysis showedmicroscopic hematuria and 3þ proteinuria, and urine culturerevealed the presence of Escherichia coli. Hemoglobin was 7.7 g/dLand leukocytosis with a left shift was noted. Urine cytology showedno abnormal cells. Renal ultrasound showed a staghorn calculus inthe right kidney (Fig. 1A). Furthermore, the diethylene-triaminepenta-acetic acid renal scan revealed nonfunction of the rightkidney, contributed for only 10% total renal function.

An intravenous urogram was not performed due to azotemiawith a serum creatinine level of 2.5 mg/dL. A high amount ofpurulent fluid coming out from the ureteral catheter was notedduring a retrograde pyelogram. A double-J stent was inserted.After conservative treatment involving blood transfusion, hydra-tion, and antibiotic administration, the patient underwent rightnephrectomy. The specimen was removed smoothly withoutdisruption.

an LLC. Open access under CC BY-NC-ND license.

Fig. 1. (A) Kidney-ureter-bladder X-ray showing a right staghorn stone; (B) grossappearance of the right kidney, measuring 13 cm � 10 cm � 8 cm. Multiple stones(arrows) were noted in the renal pelvis and calyces with marked dilatation of thecollecting system in the coronal cut surface. A polypoid and cauliflower-like tumor(arrowhead) measuring approximately 3 cm � 3 cm � 2 cm in size was observed at thelower pole of the kidney. The upper part of the dilated collecting system is filled withpurulent debris and surrounded by yellowish fatty tissue (asterisks), corresponding toxanthogranulomatous inflammation; and (C) magnified image of the cauliflower-liketumor shown in Fig. 1B.

C.-W. Tseng et al. / Urological Science 26 (2015) 69e7170

Grossly, the cystic-shaped right kidney, measuring13 cm � 10 cm � 8 cm, was dark colored and was easily dissected(Fig. 1B and C). Multiple stones were noted in the renal pelvis andcalyceswithmarked dilatation of the collecting system in the coronalcut surface. A polypoid and cauliflower-like tumor measuringapproximately 3 cm� 3 cm� 2 cm in sizewas observed at the lowerpoleof the kidney. Theupper partof thedilated collecting systemwasfilled with purulent debris and surrounded by yellowish fatty tissuecorresponding to xanthogranulomatous inflammation. The histopa-thology demonstrated high-grade (World Health Organization,

2004) papillary TCC with infiltration into the renal pelvis wall andmedulla. Nests of infiltrating transitional cells with enlarged, hyper-chromatic nuclei and pleomorphism were observed. No infiltrationwas noted in the renal cortex, hilar lymph nodes, adrenal gland, orureter (Fig. 2). XGP presented as fibrous background, cholesterolclefts, and granulomatous inflammatory infiltrate with lymphoidgerminal center formation. No SCC was found in the resected spec-imen. No carcinoma was present at the ureteral resection margin.

The patient refused further surgical treatment by ureterectomywith a bladder cuff and further alternative treatment, such aschemotherapy. Postoperative chest X-ray, technetium-99m wholebody bone scan, and pelvis-abdominal computed tomography (CT)showed no evidence of metastasis. Pathological Tumor-Node-Metastasis staging was Stage 3 (pT3N0M0).

Nine months later, the patient was readmitted with symptomsof right upper quadrant pain, abdominal fullness, nausea, vomiting,and yellowish skin lasting for 5 days. Abdominal ultrasoundshowed multiple metastatic tumors over the right lobe of the liver.Furthermore, abdominal CT revealed multiple nodal metastasis andtumor implantations in the duodenum, retrocaval space, right he-patic surface, peritoneal space, remnant right ureter stump, andurinary bladder. Endoscopic retrograde cholangiopancreatography(ERCP) showed deformity of the duodenal bulb and obstruction ofthe first portion of duodenum by a tumor. Biopsies showed meta-static urothelial carcinoma. Because the ERCP procedure wasincomplete, percutaneous transhepatic cholangiography anddrainage were performed to relieve the biliary obstruction. Furthersurgical treatment involving right ureterectomy and transurethralresection of the bladder tumor was performed, and histologicalanalysis revealed recurrent high-grade papillary TCC. Four cycles ofpostoperative palliative gemcitabine chemotherapy were planned,but the patient expired 10 days after surgery due to severe, pro-gressive hepatic encephalopathy.

3. Discussion

Staghorn calculi are primarily composed of a mixture of mag-nesium ammonium phosphate (struvite) and calcium carbonateapatite that is produced by urea-splitting organisms such as Pro-teus, Klebsiella, Pseudomonas, and Staphylococcus species. If leftuntreated, such stones can result in deterioration of renal function,XGP, life-threatening urosepsis, or even renal malignancies.1,2

Several aspects of this case are unique. First, the patient had arare combination of complete staghorn calculi, XGP, and TCC of therenal pelvis. XGP is an uncommon, aggressive inflammation of therenal parenchyma that may occur in the presence of chronicobstruction or infection.7 Renal calculi, frequently of the staghorntype, have been reported in 47e100% of cases with XGP.7 The as-sociation between XGP and renal malignancy is rarely described inliterature. One case of a patient with XGP and TCC of the renal pelviswas previously reported, but without the presence of urolithiasis.7

Long-standing staghorn calculi are associated with TCC.4 Chronic,mechanical irritation by staghorn calculi may result in squamousmetaplasia,which subsequently develops into SCC. The cause of TCCwith the presence of staghorn calculi remains unclear. However,several studies have provided evidence that TCC is associated withurinary calculi. In a prospective cohort study, patients with kidneyor ureteral stones were observed for renal pelvic/ureteral cancer.8

The gold standard procedure for TCC of the renal pelvis isnephroureterectomy with bladder cuff excision. However, our pa-tient refused this recommendation and did not return to ouroutpatient department until 9 months later.

The present case is also unique because TCC of the renal pelvismetastasized to the duodenum, which caused obstruction of thebiliary tract causing jaundice. According to Shinagare et al,9 in the

Fig. 2. (A) Photomicrograph showing areas of invasive, papillary transitional cell carcinomawith extension to the full depth of the renal parenchyma [hematoxylin and eosin (H&E),40�]; (B) note nests of infiltrating transitional cells with enlarged, hyperchromatic nuclei and pleomorphism (H&E, 400�); (C) xanthogranulomatous pyelonephritis (XGP) pre-senting as fibrous background, cholesterol clefts, and granulomatous inflammatory infiltrate with lymphoid germinal center formation (H&E, 100�); and (D) XGP showed char-acteristic lipid-laden foamy macrophages or xanthoma cells (H&E, 400�).

C.-W. Tseng et al. / Urological Science 26 (2015) 69e71 71

first study on the metastatic pattern of renal TCC in 52 patients,lymph nodes were the most common site of metastasis (75%), fol-lowed by the lung (65%), liver (54%), bone (39%), peritoneum (19%),pleura (15%), soft tissue (15%), adrenal gland (14%), and brain (8%).Here we stress the importance of radiological survey of thegastrointestinal tract in patients with upper urinary tract TCC.

The prevalence of carcinoma associated with kidney stonescausing loss of kidney function remains unclear. A high incidence ofmalignancy, including 17 cases of TCC, five cases of renal carcinoma,one case of SCC, and one case of epidermoid carcinoma, wasobserved in 24 of 47 (51%) patients diagnosed with a nonfunc-tioning kidney caused by kidney stones, who underwent ne-phrectomy, as reported by Yeh et al10; this suggests the importanceof careful pathological evaluation of surgical specimens to evaluatethe necessity of performing a more invasive surgery such asnephroureterectomy. Moreover, the possibility of upper urinarytract malignancy in patients with XGP and a nonfunctioning kidneyshould be considered. Preoperative noncontrast CT may be helpfulfor evaluating the possibility of malignancy.4 A frozen biopsycollected intraoperatively is also advised for evaluating whethernephroureterectomy with bladder cuff excision is necessary.

Conflicts of interest

The authors declare that they have no financial or non-financialconflicts of interest related to the subject matter or materials dis-cussed in the manuscript.

Sources of Funding

No funding was received for the work described in the article.

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