3.1.3. Case 3The patient was a 38-year-old woman with a family history of VHL disease and confirmed to have a missense mutation L128P in the VHL gene, who presented with multiple bilateral renal tumors. Multiple partial nephrectomies with tumor enucleations were performed. A total of 6 tumors were identified, that ranged in size from 0.4 to 6.5 cm. Of these, 4 tumors were CCRCCs, and 2 tumors showed were diagnosed as CCPRCC (tumor sizes 0.4 and 2.5 cm) (Fig. 4). Both CCPRCCs were confined to the kidney (pT1). Immunohistochemical stains were performed, which demonstrated strong expression of CK7 and CD10 in the neoplastic cells (Fig. 5). P504S was negative. In contrast, immunohistochemical stains performed on 2 separate CCRCCs demonstrated the tumor cells to be only focally positive for CK7 and diffusely positive for CD10, vimentin, and PAX-8, whereas P504S was negative.Download : Download high-res image (334KB)Download : Download full-size imageFig. 4. Patient 3: Gross appearance of the radical nephrectomy specimen showing an extensively cystic kidney. There were multiple tumors identified. The largest tumor was a 6.5 cm CCRCC located in the interpolar areas of the kidney, which showed a characteristic gross appearance for CCRCC. There were multiple additional smaller tumors including 2 separate CCPRCCs (2.5 and 0.4 cm each).