Figure 1. Atrophic right kidney, dilated left kidney with heterogeneous pelvic content (without contrast).

Figure 2. CT axial cut with heterogeneous content in left renal pelvis

Figure 3. Urotheliectomy.

Figure 4. Bladder and prostate with conic infection.

Figure 5. Right atrophic uronephrotic kidney.

Figure 6. Left kidney Bellini tumor.

Figure 7. Microscopic aspect of Bellini tumor.

Figure 8. Axial cut after urotheliectomy. No evidence of local relapse at the abdominal level.

Figure 9. Three months after urotheliectomy, no evidence of disease.

Introduction

Renal carcinoma of the Bellini pathway is a very rare histological variant of renal tumor. It was first described in 1949 and recognized with its own identity in 1986. It is an aggressive form of renal neoplasia that accounts for 1% of malignant epithelial lesions of the kidney.¹

Case Presentation

A 76-year-old male patient presented with a history of being functional monorenal due to hydronephrosis of unclear cause, chronic renal failure (usual creatinine between 1.5 and 1.7 mg/dL), with a diagnosis of upper urinary tract carcinoma in October 2020 performed by ureterorenoscopy but without biopsy at another center, and he had a history of urinary tract infections due to multidrug-resistant organisms.

He was admitted during the pandemic, presenting with gross hematuria with the presence of clots associated with intermittent dysuria and left low back pain, subsequently developing asthenia, hyporexia, and oliguria. He presented with deterioration of renal function (urea 192 mg/dL, creatinine 6.98 mg/dL) with hyponatremia (120 mEq/L), elevated lactate dehydrogenase and creatine phosphokinase (235 and 224 U/L, respectively), and positive urinary culture for multiresistant Klebsiella pneumoniae. Swab for SARS-CoV-2 was negative. CT was performed (Figures 1 and 2).

Emergency hemodialysis was performed after correction of hyponatremia and meropenem treatment. Given the possibility of a urothelial tumor in the left renal pelvis, with gross hematuria, a nonfunctioning contralateral kidney (patient on dialysis) and multiple urinary tract infections by multidrug-resistant organisms, it was decided in a multidisciplinary consultation to perform bilateral nephroureterectomy and cystoprostatectomy. This procedure (urotheliectomy) was described for the first time in 2006 and is an option for multicentric urothelial carcinomas, and we considered it as a solution for this case. The term was first coined by Berglund et al to describe a surgical procedure in which both kidneys were resected with ureters and bladder.

The surgical specimen showed an atrophic and uronephrotic right kidney, bladder with chronic inflammation, and the left kidney with a large Bellini tumor (Figures 3-7).

The patient became anephric, performing triweekly dialysis, and had a post-surgical CT at 3 months without evidence of local or distant relapses (Figures 8 and 9).

The patient died 7 months after surgery without evidence of recurrence, and without systemic treatment, after suffering a bleeding vascular brain accident.

Discussion

Bellini tumors usually mimic upper urothelium tumors (Figures 3 and 6),² so they are usually a diagnostic challenge.

Renal carcinomas of the Bellini collecting ducts are treated by surgery, systemic therapy, or both. They have an average survival time of 11 months, and only 1 patient survived more than 5 years after diagnosis.³ Most cases are similar to our patient, who managed to achieve a shorter survival of 7 months after urotheliectomy, and die from other causes without recurrence.

The best treatment available today is based on surgical resection whenever possible, usually accompanied by systemic chemotherapy with the same regimen as urothelium tumors in cases of distant disease. However, in most cases, the prognosis is usually poor despite the treatment instituted,⁴ so we agree on surgical performance and subsequent follow-up of the case presented in our study, performing urotheliectomy.

The authors of this study reported another case of urotheliectomy in 2014, where it was demonstrated once again that urotheliectomy⁵ is a useful resource in this type of patient or in multicentric urothelial tumors.⁶

Conclusions

Urotheliectomy is a recently described therapeutic resource, rarely used, and with few publications. If we assumed that the lesion was a Bellini tumor, we would have managed it the same way, since leaving the patient on dialysis and with repeated infections could lead to bladder empyema or repeated urosepsis.

Conflicts of Interest

The Authors have no competing interests to disclose.