Translocation renal cell carcinoma (RCC) is a rare aggressive malignancy in adults. A 40-year-old man presented with painless macroscopic haematuria. Initial investigations of renal ultrasound, CT scan, urine culture and urine cytology were normal. On rigid cystoscopy and pyelo-ureteroscopy, bleeding was seen from a normal-appearing right renal upper pole. An arteriovenous malformation was suspected, and laser cautery was performed. Left renal biopsy was performed due to proteinuria and suggested IgA nephropathy. The patient represented with haematuria and repeat imaging only 17 months after first presentation displayed a 9.8 cm right renal tumour with renal vein thrombus. An upper tract urothelial cancer was suspected, and the patient underwent right nephroureterectomy. Histopathology revealed translocation RCC, completely excised. Ten months postoperatively, metastasis was seen on surveillance imaging and subsequently confirmed on biopsy. The patient was referred for systemic therapy and remains alive 19 months postprocedure.
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