Simultaneous ipsilateral rhabdoid renal cell carcinoma and multifocal urothelial carcinoma of the ureter in a patient from the region of Balkan endemic nephropathy: Case report and literature review

Abstract

Introduction. Simultaneous ipsilateral coexistence of renal cell carcinoma (RCC) and upper urinary tract urothelial carcinoma (UTUC) rarely occurs. Balkan endemic nephropathy (BEN) is a chronic degenerative tubulointerstitial renal disease, strongly associated with UTUC. Case outline. А 60-year-old man from the region of BEN was referred to our clinic due to right flank pain, fever, and purulent discharge from the cutaneous fistulous opening in the right lumbar area. Multislice computed tomography urography scan showed right-side pyonephrosis and nephrocutaneous fistulous tract between the kidney and the skin in the right lumbar region. Cystoscopy detected a papillary tumor protruding from the right ureteric orifice. Right-side nephroureterectomy with bladder cuff excision was performed. Histopathological examination revealed rhabdoid RCC of the kidney and multifocal urothelial carcinoma of the ureter. Conclusion. Our case report and literature review indicate that due to rising incidence of multiple primary malignant neoplasms (MPMNs), when treating patients with RCC or UTUC, and especially those from the region of BEN, one should keep in mind the likelihood of synchronous or metachronous occurrence of these tumors.

Keywords

• rhabdoid renal cell carcinoma
• urothelial carcinoma
• balkan endemic nephropathy
• pyonephrosis
• nephrocutaneous fistula