Abstract

We report a case of pleomorphic sarcoma in a 42-year-old man with adult polycystic kidney disease [APKD]. Abdominal ultrasonography, computed tomography and MRI scans have revealed heterogenous bilateral renal cysts with a voluminous mass in the upper pole left kidney. Radical left nephrectomy with histopathologic and immunohistologic examination have confirmed the diagnosis of pleomorphic sarcoma. Sarcoma associated with adult polycystic kidney disease is extremely rare and does not have particular radiological or biological features when compared to primary renal sarcoma.

Highlights

  • Autosomal dominant polycystic kidney disease [APKD] occurs in approximately 1/500 to 1/1000 live births and is present in 10% - 12% of end-stage renal disease patients [1]

  • We report a case of pleomorphic sarcoma in a 42-year-old man with adult polycystic kidney disease [APKD]

  • Sarcoma associated with adult polycystic kidney disease is extremely rare and does not have particular radiological or biological features when compared to primary renal sarcoma

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Summary

Introduction

Autosomal dominant polycystic kidney disease [APKD] occurs in approximately 1/500 to 1/1000 live births and is present in 10% - 12% of end-stage renal disease patients [1]. It is able, like other genetic diseases, of malignant transformation [2]. Renal cell carcinoma associated with APKD has already been reported in the literature, whereas only few cases of sarcoma have been reported. We report a case of renal pleomorphic sarcoma in a patient with APKD

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