Autosomal dominant polycystic kidney disease coming up with an unusual presentation of renal cell carcinoma on its first encounter

Abstract

Autosomal polycystic kidney disease (ADPKD) is a hereditary condition characterized by expanding cysts that destroys the renal parenchyma causing ultimately the renal failure. The role of ADPKD in causing Renal cell carcinoma (RCC) is still under discussion. A 40-year-old male came with fever with chills with history of hematuria, flank pain and weight loss for 2 months. A CT urography showed Bilateral adult polycystic kidney disease and Upper pole of right kidney showing large well-defined lobulated heterogeneously enhancing mass measuring 11x10.5x11.4 cm, most likely renal cell carcinoma. Patient underwent open right radical nephrectomy. On histopathology, diagnosis was given as Papillary Renal cell carcinoma, background kidney showing features of Autosomal polycystic kidney disease. This case report comprehensively described the coexistence of Adult polycystic kidney disease with renal cell carcinoma, which is unique presentation to present with.