Staghorn calculi and xanthogranulomatous pyelonephritis associated with transitional cell carcinoma

Staghorn calculi and xanthogranulomatous pyelonephritis associated with transitional cell carcinoma

Urological Science xxx (2015) 1e3 Contents lists available at ScienceDirect Urological Science journal homepage: www.urol-sci.com Case report Stag...

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Urological Science xxx (2015) 1e3

Contents lists available at ScienceDirect

Urological Science journal homepage: www.urol-sci.com

Case report

Staghorn calculi and xanthogranulomatous pyelonephritis associated with transitional cell carcinoma Chao-Wei Tseng a, Wei-Nung Jim Chen a, Guang-Dar Juang a, Thomas I-Sheng Hwang a, b, c, * a b c

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

a r t i c l e i n f o

a b s t r a c t

Article history: Received 4 August 2014 Received in revised form 11 December 2014 Accepted 14 December 2014 Available online xxx

Untreated staghorn calculi can cause xanthogranulomatous pyelonephritis (XGP), diminished renal function, and renal malignancy. Squamous cell carcinoma (SCC) of the upper urinary tract is associated with kidney stones and chronic infection, but their association with transitional cell carcinoma (TCC) has not been proven and has rarely been reported in literature. We present a rare case of staghorn calculi and XGP associated with TCC. Copyright © 2015, Taiwan Urological Association. Published by Elsevier Taiwan LLC. All rights reserved.

Keywords: metastasis renal pelvis staghorn calculus transitional cell carcinoma xanthogranulomatous pyelonephritis

1. Introduction

2. Case Report

Staghorn calculi are large and branched stones that can fill an entire renal calyceal system. Persistent chronic irritation and/or infection by the staghorn calculi can cause xanthogranulomatous pyelonephritis (XGP), diminished renal function, or renal malignancy.1,2 The most common type of cancer associated with staghorn calculi is squamous cell carcinoma (SCC), with a varying incidence of 18e100%,3,4 followed by the rarely reported transitional cell carcinoma (TCC). TCC of the upper urinary tract is uniformly fatal unless it is treated appropriately. In a multicenter study of 10,355 patients with TCC, the 5-year cancer-specific survival rate was approximately 54%.5 TCC has a recurrence rate of 50% in the bladder6 and a high metastases rate up to 75% in the lymph nodes.7 Thus, early and proper management of TCC is essential for patient survival. Here, we report an extremely rare case of a patient who had staghorn calculi and XGP associated with TCC.

A 75-year-old female presented with dysuria, high urination frequency, and right flank pain for 5 days. She had a 4-year history of medically controlled hypertension and Stage 3 chronic kidney disease as a comorbid condition. Her family history and physical examination were noncontributory. She had not undergone any surgical procedures the previous day. The urine analysis showed microscopic hematuria and 3þ proteinuria, and urine culture revealed the presence of Escherichia coli. Hemoglobin was 7.7 g/dL and leukocytosis with a left shift was noted. Urine cytology showed no abnormal cells. Renal ultrasound showed a staghorn calculus in the right kidney (Fig. 1A). Furthermore, the diethylene-triamine penta-acetic acid renal scan revealed nonfunction of the right kidney, contributed for only 10% total renal function. An intravenous urogram was not performed due to azotemia with a serum creatinine level of 2.5 mg/dL. A high amount of purulent fluid coming out from the ureteral catheter was noted during a retrograde pyelogram. A double-J stent was inserted. After conservative treatment involving blood transfusion, hydration, and antibiotic administration, the patient underwent right nephrectomy. The specimen was removed smoothly without disruption.

* Corresponding author. Division of Urology, Department of Surgery, Shin-Kong Wu Ho-Su Memorial Hospital, No. 95, Wen Chang Road, Shih Lin District, Taipei City, Taiwan. E-mail address: [email protected] (T.I.-S. Hwang).

http://dx.doi.org/10.1016/j.urols.2014.12.006 1879-5226/Copyright © 2015, Taiwan Urological Association. Published by Elsevier Taiwan LLC. All rights reserved.

Please cite this article in press as: Tseng C-W, et al., Staghorn calculi and xanthogranulomatous pyelonephritis associated with transitional cell carcinoma, Urological Science (2015), http://dx.doi.org/10.1016/j.urols.2014.12.006

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C.-W. Tseng et al. / Urological Science xxx (2015) 1e3

(World Health Organization, 2004) papillary TCC with infiltration into the renal pelvis wall and medulla. Nests of infiltrating transitional cells with enlarged, hyperchromatic nuclei and pleomorphism were observed. No infiltration was noted in the renal cortex, hilar lymph nodes, adrenal gland, or ureter (Fig. 2). XGP presented as fibrous background, cholesterol clefts, and granulomatous inflammatory infiltrate with lymphoid germinal center formation. No SCC was found in the resected specimen. No carcinoma was present at the ureteral resection margin. The patient refused further surgical treatment by ureterectomy with a bladder cuff and further alternative treatment, such as chemotherapy. Postoperative chest X-ray, technetium-99m whole body bone scan, and pelvis-abdominal computed tomography (CT) showed no evidence of metastasis. Pathological Tumor-NodeMetastasis staging was Stage 3 (pT3N0M0). Nine months later, the patient was readmitted with symptoms of right upper quadrant pain, abdominal fullness, nausea, vomiting, and yellowish skin lasting for 5 days. Abdominal ultrasound showed multiple metastatic tumors over the right lobe of the liver. Furthermore, abdominal CT revealed multiple nodal metastasis and tumor implantations in the duodenum, retrocaval space, right hepatic surface, peritoneal space, remnant right ureter stump, and urinary bladder. Endoscopic retrograde cholangiopancreatography (ERCP) showed deformity of the duodenal bulb and obstruction of the first portion of duodenum by a tumor. Biopsies showed metastatic urothelial carcinoma. Because the ERCP procedure was incomplete, percutaneous transhepatic cholangiography and drainage were performed to relieve the biliary obstruction. Further surgical treatment involving right ureterectomy and transurethral resection of the bladder tumor was performed, and histological analysis revealed recurrent high-grade papillary TCC. Four cycles of postoperative palliative gemcitabine chemotherapy were planned, but the patient expired 10 days after surgery due to severe, progressive hepatic encephalopathy. 3. Discussion

Fig. 1. (A) Kidney-ureter-bladder X-ray showing a right staghorn stone; (B) gross appearance 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 the collecting 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 the lower pole of the kidney. The upper part of the dilated collecting system is filled with purulent debris and surrounded by yellowish fatty tissue (asterisks), corresponding to xanthogranulomatous inflammation; and (C) magnified image of the cauliflower-like tumor shown in Fig. 1B.

Grossly, the cystic-shaped right kidney, measuring 13 cm  10 cm  8 cm, was dark colored and was easily dissected (Fig. 1B and C). Multiple stones were noted in the renal pelvis and calyces with marked dilatation of the collecting system in the coronal cut surface. A polypoid and cauliflower-like tumor measuring approximately 3 cm  3 cm  2 cm in size was observed at the lower pole of the kidney. The upper part of the dilated collecting system was filled with purulent debris and surrounded by yellowish fatty tissue corresponding to xanthogranulomatous inflammation. The histopathology demonstrated high-grade

Staghorn calculi are primarily composed of a mixture of magnesium ammonium phosphate (struvite) and calcium carbonate apatite that is produced by urea-splitting organisms such as Proteus, Klebsiella, Pseudomonas, and Staphylococcus species. If left untreated, 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 a rare combination of complete staghorn calculi, XGP, and TCC of the renal pelvis. XGP is an uncommon, aggressive inflammation of the renal parenchyma that may occur in the presence of chronic obstruction or infection.7 Renal calculi, frequently of the staghorn type, have been reported in 47e100% of cases with XGP.7 The association between XGP and renal malignancy is rarely described in literature. One case of a patient with XGP and TCC of the renal pelvis was 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 squamous metaplasia, which subsequently develops into SCC. The cause of TCC with the presence of staghorn calculi remains unclear. However, several studies have provided evidence that TCC is associated with urinary calculi. In a prospective cohort study, patients with kidney or ureteral stones were observed for renal pelvic/ureteral cancer.8 The gold standard procedure for TCC of the renal pelvis is nephroureterectomy with bladder cuff excision. However, our patient refused this recommendation and did not return to our outpatient department until 9 months later.

Please cite this article in press as: Tseng C-W, et al., Staghorn calculi and xanthogranulomatous pyelonephritis associated with transitional cell carcinoma, Urological Science (2015), http://dx.doi.org/10.1016/j.urols.2014.12.006

C.-W. Tseng et al. / Urological Science xxx (2015) 1e3

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Fig. 2. (A) Photomicrograph showing areas of invasive, papillary transitional cell carcinoma with 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) presenting as fibrous background, cholesterol clefts, and granulomatous inflammatory infiltrate with lymphoid germinal center formation (H&E, 100); and (D) XGP showed characteristic lipid-laden foamy macrophages or xanthoma cells (H&E, 400).

The present case is also unique because TCC of the renal pelvis metastasized to the duodenum, which caused obstruction of the biliary tract causing jaundice. According to Shinagare et al,9 in the first study on the metastatic pattern of renal TCC in 52 patients, lymph nodes were the most common site of metastasis (75%), followed 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 the gastrointestinal tract in patients with upper urinary tract TCC. The prevalence of carcinoma associated with kidney stones causing loss of kidney function remains unclear. A high incidence of malignancy, including 17 cases of TCC, five cases of renal carcinoma, one case of SCC, and one case of epidermoid carcinoma, was observed in 24 of 47 (51%) patients diagnosed with a nonfunctioning kidney caused by kidney stones, who underwent nephrectomy, as reported by Yeh et al10; this suggests the importance of careful pathological evaluation of surgical specimens to evaluate the necessity of performing a more invasive surgery such as nephroureterectomy. Moreover, the possibility of upper urinary tract malignancy in patients with XGP and a nonfunctioning kidney should be considered. Preoperative noncontrast CT may be helpful for evaluating the possibility of malignancy.4 A frozen biopsy collected intraoperatively is also advised for evaluating whether nephroureterectomy with bladder cuff excision is necessary.

Conflicts of interest The authors declare that they have no financial or non-financial conflicts of interest related to the subject matter or materials discussed in the manuscript. References 1. Koga S, Arakaki Y, Matsuoka M, Ohyama C. Staghorn calculidlong-term results of management. Br J Urol 1991;68:122e4. 2. Vargas AD, Bragin SD, Mendez R. Staghorn calculis: its clinical presentation, complications and management. J Urol 1982;127:860e2. 3. Blacher EJ, Johnson DE, Abdul-Karim FW, Ayala AG. Squamous cell carcinoma of renal pelvis. Urology 1985;25:124e6. 4. Raghavendran M, Rastogi A, Dubey D, Chaudhary H, Kumar A, Srivastava A, et al. Stones associated renal pelvic malignancies. Indian J Cancer 2003;40:108e12. 5. Visser O, Adolfsson J, Rossi S, Verne J, Gatta G, Maffezzini M, et al. Incidence and survival of rare urogenital cancers in Europe. Eur J Cancer 2012;48:456e64. 6. Olgac S, Mazumdar M, Dalbagni G, Reuter VE. Urothelial carcinoma of the renal pelvis: a clinicopathologic study of 130 cases. Am J Surg Pathol 2004;28:1545e52. 7. Val-Bernal JF, Castro F. Xanthogranulomatous pyelonephritis associated with transitional cell carcinoma of the renal pelvis. Urol Int 1996;57:240e5. 8. Kaufmann JM, Fam B, Jacobs SC, Gabilondo F, Yalla S, Kane JP, et al. Bladder cancer and squamous metaplasia in spinal cord injury patients. J Urol 1977;118:967e71. 9. Shinagare AB, Fennessy FM, Ramaiya NH, Jagannathan JP, Taplin ME, Van den Abbeele AD. Urothelial cancers of the upper urinary tract: metastatic pattern and its correlation with tumor histopathology and location. J Comput Assist Tomogr 2011;35:217e22. 10. Yeh CC, Lin TH, Wu HC, Chang CH, Chen CC, Chen WC. A high association of upper urinary tract transitional cell carcinoma with nonfunctioning kidney caused by stone disease in Taiwan. Urol Int 2007;79:19e23.

Please cite this article in press as: Tseng C-W, et al., Staghorn calculi and xanthogranulomatous pyelonephritis associated with transitional cell carcinoma, Urological Science (2015), http://dx.doi.org/10.1016/j.urols.2014.12.006