In 1978, a 24-year-old man who had been managed by hemodialysis for 7 years was referred to our department for emergency nephrectomy as his “autosomal dominant polycystic kidney disease” was infected, and the condition had become uncontrollable (Fig. 2). A large hematoma was present in the resected lower pole of the right kidney, and multiple small cysts were observed in both kidneys. The pathology department reported autosomal dominant polycystic kidney disease (ADPKD) complicated by hematoma (Fig. 3). However, careful inquiry into the patient’s history revealed that he had undergone renal biopsy 7 months before the initiation of dialysis, and had been diagnosed as having rapidly progressive glomerulonephritis (Figs. 4 and 5). This reminded me of something I had learned in 1972 while I was studying in the United States: “Cysts eventually develop in all end-stage kidneys.” Reviewing the literature from that time, I came across an autopsy report by Dunnill et al. [1] (Fig. 6) published in 1977. This described cysts complicated by renal cell carcinoma (RCC). I therefore speculated that our patient had initially had rapidly progressive glomerulonephritis and thereafter had developed acquired renal cysts, which were then complicated by renal cell carcinoma, and that the hematoma was due to bleeding of the renal cell carcinoma. I requested that the pathology department reevaluate the case. The reevaluation disclosed a papillary renal cell carcinoma consisting of a clear cell carcinoma and a granular cell carcinoma on the hematoma wall (Fig. 7). This was my first clinical case of acquired cystic disease of the kidney and renal cell carcinoma, and also the first case in the world.


Renal Cell Carcinoma Renal Biopsy Autosomal Dominant Polycystic Kidney Disease Renal Cyst Clear Cell Carcinoma 
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© Springer 2007

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