Renal cell carcinoma in adult polycystic kidney disease is of rare occurrence and poses a diagnostic and therapeutic challenge. We have treated three such patients in our department. One was male and two were females ranging in age from 20 to 60 years. All were diagnosed preoperatively with ultrasonography or contrast-enhanced CT. Radical nephrectomy was performed in all patients. During the follow-up no patient had recurrence in the contralateral kidney. One patient had local recurrence in the renal fossa and was treated with local radiotherapy and immunotherapy. In the past due to difficulty in diagnosis, invasive investigations like angiography were recommended and prophylactic contralateral nephrectomy was often undertaken. With the availability of reliable noninvasive investigations these patients can be diagnosed accurately and the contralateral kidney saved in most cases.

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