Progression of hydronephrosis in end-stage renal disease as a potential indicator of underlying upper urinary tract tumors: a report of a rare case

Abstract

Upper tract urothelial carcinoma (UTUC) is a rare malignancy with a higher incidence in patients with end-stage renal disease (ESRD), particularly in regions with specific environmental risk factors. The diagnosis of UTUC in patients with ESRD remains challenging because of certain limitations inherent to current urine cytology techniques and imaging quality. Herein, we report the case of a 72-year-old woman with a history of bladder urothelial carcinoma and ESRD who was receiving regular hemodialysis and was incidentally diagnosed with high-grade multifocal UTUC following a nephrectomy procedure to treat symptomatic hydronephrosis. Despite routine cystoscopic surveillance and unremarkable cytological findings, progressive hydronephrosis was noted on serial imaging. Surgical intervention was performed as the patient’s symptoms worsened progressively, after which pathological examination confirmed the presence of UTUC with renal parenchymal invasion. This case highlights the notion that UTUC should be considered as a differential diagnosis in patients with ESRD exhibiting symptomatic hydronephrosis of an unexplained etiology.

Plain language summary

Worsening kidney swelling in advanced kidney disease may signal hidden urinary tract cancer: a report of a rare case

Upper tract urothelial carcinoma (UTUC) is a rare type of cancer that affects the lining of the upper urinary tract. It is more common in people with end-stage kidney disease (ESRD), especially in areas with certain environmental risks. However, diagnosing this cancer in patients with ESRD can be difficult because common tests like urine analysis and imaging don’t always detect it clearly. In this report, we describe the case of a 72-year-old woman who had a history of bladder cancer and was on regular dialysis for ESRD. She was unexpectedly found to have UTUC after having surgery to remove a kidney due to worsening kidney swelling (hydronephrosis) that caused symptoms. Even though her regular bladder exams and urine tests showed no signs of cancer, scans showed that the swelling in her kidney was getting worse. Surgery was eventually done because her symptoms increased, and tests on the removed kidney revealed high-grade UTUC that had spread into the kidney tissue. This case shows that in patients with ESRD, unexplained and worsening kidney swelling should raise concern for possible UTUC, even if other test results appear normal.

Introduction

Upper tract urothelial carcinoma (UTUC) arises from the urothelial cells that line the renal pelvis and ureters. It is relatively rare, accounting for only 5%–10% of all urothelial carcinomas. Its incidence varies geographically, with males being more frequently affected than females (male-to-female ratio, ~2:1). Its etiology is similar to that of bladder cancer, as both include smoking, exposure to aromatic amines, chronic inflammation, and aristolochic acid ingestion. ¹ In Taiwan, exposure to aristolochic acid and arsenic in water represent the two major risk factors for UTUC. End-stage renal disease (ESRD), which necessitates the use of regular dialysis in patients who suffer from it, has been reported to represent a significant risk factor for the development of UTUC. Diagnosing UTUC is more challenging in patients with ESRD because of more limited access to urine specimens, constraints related to image quality on computed tomography (CT) scans, and a difficult endoscopic approach. However, because of the poor prognoses of UTUCs with grades of ⩾T2, early diagnosis and surgical management are essential to improving survival outcomes in patients. Herein, we present the case of a patient with ESRD who was incidentally diagnosed with advanced-stage UTUC after undergoing a nephrectomy procedure to treat progressive and symptomatic hydronephrosis.

Case report

A 72-year-old woman presented to the hospital with dull pain in her left flank that had persisted for several months, accompanied by a palpable mass in the region. She was a non-smoker, a housewife, and lived outside the region of arsenic contamination. No regular use of herbal medicines was reported by the patient. She had a prior history of stage I bladder urothelial carcinoma (cT1N0M0) following transurethral resection of a bladder tumor 3 years prior, as well as ESRD for which she had been receiving regular hemodialysis (QW 1.3.5) with anuria. Her previous bladder tumor had been located near the left ureterovesical junction, and severe scarring with obstruction of the left ureteral orifice had been observed during regular cystoscopic follow-ups. Washed cytology samples were collected during surveillance cystoscopy, but did not show any remarkable findings. Her final cystoscopy had been performed 3 months prior. Magnetic resonance imaging (MRI) of her bladder was performed annually, which had recently revealed progressive left hydronephrosis during several serial checkups (Figure 1(a) and (b)). She denied having fever, chills, urgency, or hematuria. Physical examination revealed a palpable, soft, and non-tender mass on the left flank. A hemogram showed a white blood cell count of 5.02 × 10³/μL, a neutrophil proportion of 71.3%, a lymphocyte proportion of 19.3%, and anemia (hemoglobin concentration, 8.8 g/dL). Biochemical analysis revealed mild hyponatremia (sodium concentration, 133 mmol/L). CT of the abdomen and pelvis without contrast-enhancement revealed left severe hydroureteronephrosis, as well as an elevated radio-density of pelvic content (15 Hounsfield units) versus the bladder urine (3 Hounsfield units). Mild mural thickness was observed over the upper pole of the left kidney; however, no perirenal fat stranding or regional lymphadenopathy was noted (Figure 2). Elective left laparoscopic nephroureterectomy was performed to treat the patient’s symptomatic hydronephrosis, after which tinged blood and pus-like contents were observed in the resected ureteral specimen.

Figure 1.

(a) Left atrophic kidney (red arrow) observed on magnetic resonance image at the time of bladder cancer diagnosis. (b) Progressive hydronephrosis (red arrow) was observed 1 year later.

Figure 2.

Left hydronephrosis with higher radiodensity content (15 Hounsfield units) compared to urine in bladder (3 Hounsfield units), mild mural thickness observed over the left upper pole of the kidney (red arrow).

Pathological examination revealed a multifocal, high-grade urothelial carcinoma of the left renal pelvis with renal parenchymal invasion. No tumor involvement was identified at the ureteral or bladder cuff margins (Figure 3). Chest to pelvis CT was performed at 1 month, revealing no evidence of local recurrence or distant metastases. Adjuvant platinum-based chemotherapy or nivolumab was recommended due to the patient’s adverse pathological T stage; however, neither treatment option was administered owing to the patient’s renal impairment and economic considerations. Close follow-up imaging was initiated thereafter. Adjuvant intravesical chemotherapy was not administered owing to the patient’s small bladder capacity. At the time of writing this report, no local recurrence or distant metastases have been detected at the patient’s 6-month follow-up.

Figure 3.

High-grade invasive urothelial carcinoma involving renal parenchyma (yellow arrow).

Discussion

Chronic kidney disease (CKD) is related to a 1.63× higher risk of developing UTUC. ² ESRD can also significantly increase the risk of both UTUC and lower tract urothelial carcinoma. Female patients with ESRD have been reported to exhibit a 9–18× higher risk, whereas males generally have a 4–14× higher risk. ³ Urine cytology and cystoscopy have limited sensitivities for diagnosing UTUC in patients receiving dialysis; therefore, CT and MRI are more promising approaches for detecting and staging this cancer type. ⁴

CKD of stages 4–5 (estimated glomerular filtration rate, <30 mL/min) has been associated with worse overall survival outcomes and a higher probability of contralateral recurrence in patients with UTUC. Careful management and monitoring are therefore recommended in this patient demographic.^5,6 Currently, routine prophylactic nephroureterectomy or cystectomy is not recommended in patients with UC and uremia. ⁷ Nevertheless, the presence of hydronephrosis in the native kidney has been significantly associated with UTUC in patients who received kidney transplants. ⁸

Based on our experience with this patient, we recommend that clinicians give careful consideration to the possibility of UTUC in patients with a history of bladder cancer and progressive hydronephrosis.

Conclusion

UTUC remains a diagnostic challenge in patients with ESRD, as this malignancy is particularly difficult to assess using endoscopic, cytological, or imaging techniques. In patients with symptomatic hydronephrosis of an unexplained etiology, UTUC should be carefully considered as a differential diagnosis, and early surgical intervention may prove beneficial.

Supplemental Material

sj-pdf-1-tau-10.1177_17562872251393081 – Supplemental material for Progression of hydronephrosis in end-stage renal disease as a potential indicator of underlying upper urinary tract tumors: a report of a rare case

Supplemental material, sj-pdf-1-tau-10.1177_17562872251393081 for Progression of hydronephrosis in end-stage renal disease as a potential indicator of underlying upper urinary tract tumors: a report of a rare case by Pai-Yu Cheng, Shiu-Dong Chung and Chin-Fong Au in Therapeutic Advances in Urology