Transitional cell carcinoma: A rare development in congenital ureteropelvic junction obstruction kidney.

A 54-year-old male chronic smoker presented with hematuria 13 years after being diagnosed with an asymptomatic left non-functioning kidney secondary to congenital ureteropelvic junction obstruction. On evaluation, he was diagnosed with renal collecting system mass occupying the lower pole region, for which radical nephroureterectomy with bladder cuff excision and nodal dissection was carried out. Histopathology revealed upper tract transitional cell carcinoma.

INTRODUCTION

A 54-year-old male, chronic smoker was diagnosed with an asymptomatic non-functioning left kidney secondary to congenital ureteropelvic junction obstruction (UPJO) during routine health check up 13 years earlier. He refused surgical intervention and was lost to follow-up till 10 months back, when he presented with gross painless hematuria. Physical examination revealed a 15 cm × 15 cm left renal lump, urinalysis showed 100 RBCs/HPF and urine cytology was negative for malignant cells. Computed tomography (CT) urography revealed left gross hydronephrosis with abrupt cut-off at PUJ with thinned out renal parenchyma and an 8 cm × 7 cm × 6 cm enhancing soft tissue mass occupying the collecting system in the lower pole region [Figure 1]. Radical nephroureterectomy with bladder cuff excision was performed with lymph node dissection from the renal hilum to the aortic bifurcation [Figure 2 left]. Histopathology revealed high-grade transitional cell carcinoma (TCC) (stage pT4, N0, M0) [Figure 2 right]. The patient is asymptomatic on follow-up.

Figure 1

Computed tomography scan demonstrating renal collecting system tumor; left – coronal view, right – sagittal view

Figure 2

Radical nephroureterectomy specimen; left – opened-up specimen showing tumor, right – high-grade transitional cell tumor present as disorganized sheets of cells with a high nuclear cytoplasmic ratio

Upper urinary tract TCC was reported in non-functioning kidney secondary to (a) Stone disease and (b) congenital UPJO with calcified renal pelvis.[12] While TCC/squamous cell carcinoma of the renal pelvis presenting as UPJO is well documented, TCC developing in a kidney with UPJO is extremely rare, with only few cases having been reported.[345]