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Presented by Pedram Argani, M.D. and prepared by Carol Allan, M.D.
Case 6: Renal mass in a 53-year-old male with autosomal dominant polycystic kidney disease.
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1. Question
Week 76: Case 6
Renal mass in a 53-year-old male with autosomal dominant polycystic kidney disease./images/pa6a.jpg
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/images/pa6c.jpgCorrect
Answer: Renal cell carcinoma, clear cell type
Histology: The lesion is composed of a nested proliferation of clear cells with a prominent capillary vascular component. The cytoplasm lacks a foamy or granular quality and appears virtually empty. The nuclei are hyperchromatic, and would qualify as Fuhrman grade 2. Significant papillary growth is not identified.
Discussion: Xanthogranulomatous polynephritis is a mimic of clear cell renal cell carcinoma, both histologically and clinically. One would expect more inflammatory component, and more finely granular cytoplasm with less discrete cell borders as is typical of histiocytic lesions. The current lesion has clear cytoplasm and lacks papillary architecture, which excludes papillary renal cell carcinoma. An adrenal cortical lesion should demonstrate fine bubbly cytoplasm with nuclear indentation, which is typical of cells containing intracytoplasmic lipid.
End stage renal disease is risk factor for the development of renal cell carcinoma, and a disproportional number of these are papillary renal cell carcinomas (48% versus 10% in the baseline population). Long-term dialysis is thought to pose a risk of acquired cystic disease, which leads to tubulopapillary hyperplasia, and the eventual development of renal carcinoma. Interestingly, autosomal dominant polycystic kidney disease does not appear itself to be associated with a significantly increased risk of renal cell carcinoma. However, in a patient with end-stage renal disease as a consequence of the polycystic kidney disease, the patient is set up to develop tubulopapillary hyperplasia which predisposes to carcinoma.
Incorrect
Answer: Renal cell carcinoma, clear cell type
Histology: The lesion is composed of a nested proliferation of clear cells with a prominent capillary vascular component. The cytoplasm lacks a foamy or granular quality and appears virtually empty. The nuclei are hyperchromatic, and would qualify as Fuhrman grade 2. Significant papillary growth is not identified.
Discussion: Xanthogranulomatous polynephritis is a mimic of clear cell renal cell carcinoma, both histologically and clinically. One would expect more inflammatory component, and more finely granular cytoplasm with less discrete cell borders as is typical of histiocytic lesions. The current lesion has clear cytoplasm and lacks papillary architecture, which excludes papillary renal cell carcinoma. An adrenal cortical lesion should demonstrate fine bubbly cytoplasm with nuclear indentation, which is typical of cells containing intracytoplasmic lipid.
End stage renal disease is risk factor for the development of renal cell carcinoma, and a disproportional number of these are papillary renal cell carcinomas (48% versus 10% in the baseline population). Long-term dialysis is thought to pose a risk of acquired cystic disease, which leads to tubulopapillary hyperplasia, and the eventual development of renal carcinoma. Interestingly, autosomal dominant polycystic kidney disease does not appear itself to be associated with a significantly increased risk of renal cell carcinoma. However, in a patient with end-stage renal disease as a consequence of the polycystic kidney disease, the patient is set up to develop tubulopapillary hyperplasia which predisposes to carcinoma.