APKD & Renal Cell Carcinoma?

[SeminoleFan3]

I had a Kaplan question today that asked, basically, what someone with APKD was at a higher risk for. It came down to subarachnoid hemorrhage (Berry aneurysms) or renal cell carcinoma. While I put the former, which ended up being right, Kaplan went on to say that those with APKD aren't at an increase risk for RCC. Goljan, however (pg 426) says that APKD is a risk factor for RCC.
Ideas?

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[calespine]

According to first aid (take with grain of salt), on page 90 and 449, there is no mention of any association with renal cell carcinoma.

Edit- there is some association based on the literature, but I guess the distinction is that the RCC is more associated with the ESRF that results from APKD--it's a fine line. So the incidence of RCC is no higher in APKD than in other acquired renal cystic or ESRF disorders. Main point- relatively speaking, the primary associations (higher risk) with APKD are polycystic liver dz, berry aneurysms, and mitral valve prolapse---much weaker assocation (lower risk in reference to the qbank q)=RCC.

 

[sendwich]

According to first aid (take with grain of salt), on page 90 and 449, there is no mention of any association with renal cell carcinoma.

Edit- there is some association based on the literature, but I guess the distinction is that the RCC is more associated with the ESRF that results from APKD--it's a fine line. So the incidence of RCC is no higher in APKD than in other acquired renal cystic or ESRF disorders. Main point- relatively speaking, the primary associations (higher risk) with APKD are polycystic liver dz, berry aneurysms, and mitral valve prolapse---much weaker assocation (lower risk in reference to the qbank q)=RCC.

could you explain why? thx

 

[calespine]

As far as I know, the mechanism behind APKD isn't entirely known, but it's hypothesized that the cystic formations (renal and extrarenal) are due to abnormal epithelial cell differentiation and/or extracellular matrix function. Which leads to the widespread vascular/membranous abnormalities seen in

• Cerebral aneurysms
• Hepatic cysts
• Cardiac valve disease
• Colonic diverticula
• Abdominal wall and inguinal hernia

 

[scube]

could you explain why? thx

defect in the protein polycystin(function unclear but is a cell membrane protein) which is found in the kidney(renal cysts), biliary epithelium (liver cysts), blood vessels(berry aneurism although goljan says it is a lack of a layer(lamina densa) at these junctions combined with hypertension, I think both can be right) also some heart valve issues due to distribution of polycysten in the valves. This is from robbins.

Hope this helps.