Clear Cell Papillary Renal Cell Carcinoma–like Tumors in Patients With Von Hippel-Lindau Disease Are Unrelated to Sporadic Clear Cell Papillary Renal Cell Carcinoma

2013 ◽  
Vol 37 (8) ◽  
pp. 1131-1139 ◽  
Author(s):  
Sean R. Williamson ◽  
Shaobo Zhang ◽  
John N. Eble ◽  
David J. Grignon ◽  
Guido Martignoni ◽  
...  
Radiology ◽  
2020 ◽  
Vol 295 (3) ◽  
pp. E10-E10
Author(s):  
Faraz Farhadi ◽  
Moozhan Nikpanah ◽  
Anna K. Paschall ◽  
Ahmad Shafiei ◽  
Ashkan Tadayoni ◽  
...  

2005 ◽  
Vol 5 ◽  
pp. 9-10 ◽  
Author(s):  
Christopher Williams ◽  
McClellan Walther

Differentiating between clear cell neuroendocrine tumor (NET) of the pancreas and renal cell carcinoma (RCC) metastatic to the pancreas can be challenging in patients with von Hippel-Lindau disease (VHL). The clear cell features of both NET and RCC in VHL patients may lead to misdiagnosis, inaccurate staging, and alternative treatment. We present a patient in which this occurred. As clear cell NETs closely resembling metastatic RCC are distinctive neoplasms of VHL and metastatic RCC to the pancreas in the VHL population is rare, careful pathologic examination should be performed prior to subjecting patients to definitive surgical or medical therapies.


Radiology ◽  
2020 ◽  
Vol 295 (3) ◽  
pp. 583-590 ◽  
Author(s):  
Faraz Farhadi ◽  
Moozhan Nikpanah ◽  
Anna K. Paschall ◽  
Ahmad Shafiei ◽  
Ashkan Tadayoni ◽  
...  

2021 ◽  
Author(s):  
Jie Wang ◽  
Lei Zhang ◽  
Jianhui Qiu ◽  
Ziao Li ◽  
Yucai Wu ◽  
...  

Abstract Purpose: To compare the tumor growth kinetics between sporadic clear cell renal cell carcinoma (ccRCC) and Von Hippel-Lindau disease-associated renal cell carcinoma (VHL-associated RCC). To analyze predictive markers for the growth rate of these two types of RCC.Methods: The clinical data of patients with renal tumors who received active surveillance were collected retrospectively. Immunohistochemical staining was utilized to analyze the expression levels of VHL, PBRM1, H3K36me3, and BAP1 in the postoperative specimens.Results: The age of the VHL group was significantly younger than that of the sporadic group (P<0.0001). The mean linear growth rate (LGR) was significantly faster in the sporadic group (P=0.0004). The tumors of those in the sporadic group tended to have a higher histologic grade (P=0.0011). In the sporadic group, tumor histologic grade was an independent predictor for rapid mean LGR (P=0.0022). In the VHL group, tumor initial MTD was the only independent predictor for rapid mean LGR (P<0.0001). Tumors with low VHL expression and negative PBRM1 expression showed a faster growth rate in the sporadic group (P=0.001 and P=0.008, respectively). The expression levels of the 4 biomarkers showed no impact on the tumor growth rate in the VHL group.Conclusion: Sporadic ccRCC grew faster than VHL-associated RCC. High histologic grade, low VHL expression and negative PBRM1 expression were predictors of faster growth in sporadic ccRCC. A large initial MTD was a predictor of faster growth for VHL-associated RCC.


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