scholarly journals Natural history of Von Hippel–Lindau disease-associated and sporadic clear cell renal cell carcinoma: a comparative study

2021 ◽  
Author(s):  
Jie Wang ◽  
Lei Zhang ◽  
Jianhui Qiu ◽  
Ziao Li ◽  
Yucai Wu ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Natural History ◽  
Comparative Study ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Clear Cell ◽  
Cell Renal Cell Carcinoma ◽  
Von Hippel Lindau ◽  
History Of ◽  
Von Hippel Lindau Disease

scholarly journals Clear Cell Renal Cell Carcinoma Growth Correlates with Baseline Diffusion-weighted MRI in Von Hippel–Lindau Disease

Radiology ◽  
2020 ◽  
Vol 295 (3) ◽  
pp. E10-E10
Author(s):  
Faraz Farhadi ◽  
Moozhan Nikpanah ◽  
Anna K. Paschall ◽  
Ahmad Shafiei ◽  
Ashkan Tadayoni ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Clear Cell ◽  
Diffusion Weighted Mri ◽  
Cell Renal Cell Carcinoma ◽  
Von Hippel Lindau ◽  
Diffusion Weighted ◽  
Von Hippel Lindau Disease

scholarly journals Clear Cell Renal Cell Carcinoma Growth Correlates with Baseline Diffusion-weighted MRI in Von Hippel–Lindau Disease

Radiology ◽  
2020 ◽  
Vol 295 (3) ◽  
pp. 583-590 ◽  
Author(s):  
Faraz Farhadi ◽  
Moozhan Nikpanah ◽  
Anna K. Paschall ◽  
Ahmad Shafiei ◽  
Ashkan Tadayoni ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Clear Cell ◽  
Diffusion Weighted Mri ◽  
Cell Renal Cell Carcinoma ◽  
Von Hippel Lindau ◽  
Diffusion Weighted ◽  
Von Hippel Lindau Disease

scholarly journals Natural History of Von Hippel-Lindau Disease-associated and Sporadic Clear Cell Renal Cell Carcinoma: a Comparative Study

2021 ◽  
Author(s):  
Jie Wang ◽  
Lei Zhang ◽  
Jianhui Qiu ◽  
Ziao Li ◽  
Yucai Wu ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Growth Rate ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Clear Cell ◽  
Histologic Grade ◽  
Cell Renal Cell Carcinoma ◽  
Sporadic Group ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease

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.

scholarly journals Rho-associated kinase 1 inhibition is synthetically lethal with von Hippel-Lindau deficiency in clear cell renal cell carcinoma

Oncogene ◽  
2016 ◽  
Vol 36 (8) ◽  
pp. 1080-1089 ◽  
Author(s):  
J M Thompson ◽  
Q H Nguyen ◽  
M Singh ◽  
M W Pavesic ◽  
I Nesterenko ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Clear Cell ◽  
Cell Renal Cell Carcinoma ◽  
Von Hippel Lindau ◽  
Synthetically Lethal

scholarly journals Targeting β2-Adrenergic Receptors Shows Therapeutical Benefits in Clear Cell Renal Cell Carcinoma from Von Hippel–Lindau Disease

2020 ◽  
Vol 9 (9) ◽  
pp. 2740
Author(s):  
Virginia Albiñana ◽  
Eunate Gallardo-Vara ◽  
Isabel de Rojas-P ◽  
Lucia Recio-Poveda ◽  
Tania Aguado ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Clear Cell ◽  
Cell Renal Cell Carcinoma ◽  
Von Hippel Lindau ◽  
Gene And Protein Expression ◽  
Ici 118,551

Von Hippel–Lindau (VHL), is a rare autosomal dominant inherited cancer in which the lack of VHL protein triggers the development of multisystemic tumors such us retinal hemangioblastomas (HB), CNS-HB, and clear cell renal cell carcinoma (ccRCC). ccRCC ranks third in terms of incidence and first in cause of death. Standard systemic therapies for VHL-ccRCC have shown limited response, with recurrent surgeries being the only effective treatment. Targeting of β2-adrenergic receptor (ADRB) has shown therapeutic antitumor benefits on VHL-retinal HB (clinical trial) and VHL-CNS HB (in vitro). Therefore, the in vitro and in vivo antitumor benefits of propranolol (ADRB-1,2 antagonist) and ICI-118,551 (ADRB-2 antagonist) on VHL−/− ccRCC primary cultures and 786-O tumor cell lines have been addressed. Propranolol and ICI-118,551 activated apoptosis inhibited gene and protein expression of HIF-2α, CAIX, and VEGF, and impaired partially the nuclear internalization of HIF-2α and NFĸB/p65. Moreover, propranolol and ICI-118,551 reduced tumor growth on two in vivo xenografts. Finally, ccRCC patients receiving propranolol as off-label treatment have shown a positive therapeutic response for two years on average. In summary, propranolol and ICI-118,551 have shown antitumor benefits in VHL-derived ccRCC, and since ccRCCs comprise 63% of the total RCCs, targeting ADRB2 becomes a promising drug for VHL and other non-VHL tumors.

Significance of PI3K signalling pathway in clear cell renal cell carcinoma in relation to VHL and HIF status

2020 ◽  
pp. jclinpath-2020-206693
Author(s):  
Raviprakash Tumkur Sitaram ◽  
Maréne Landström ◽  
Göran Roos ◽  
Börje Ljungberg
Keyword(s):  
Renal Cell Carcinoma ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Clear Cell ◽  
Tumour Progression ◽  
Recent Decade ◽  
Signalling Pathway ◽  
Signalling Pathways ◽  
Cell Renal Cell Carcinoma ◽  
Von Hippel Lindau

Renal cell carcinoma (RCC) includes diverse tumour types characterised by various genetic abnormalities. The genetic changes, like mutations, deletions and epigenetic alterations, play a crucial role in the modification of signalling networks, tumour pathogenesis and prognosis. The most prevalent RCC type, clear cell RCC (ccRCC), is asymptomatic in the early stages and has a poorer prognosis compared with the papillary and the chromophobe types RCCs. Generally, ccRCC is refractory to chemotherapy and radiation therapy. Loss of von Hippel-Lindau (VHL) gene and upregulation of hypoxia-inducible factors (HIF), the signature of most sporadic ccRCC, promote multiple growth factors. Hence, VHL/HIF and a variety of pathways, including phosphatase and TEnsin homolog on chromosome 10/phosphatidylinositol-3-kinase (PI3K)/AKT, are closely connected and contribute to the ontogeny of ccRCC. In the recent decade, multiple targeting agents have been developed based on blocking major signalling pathways directly or indirectly involved in ccRCC tumour progression, metastasis, angiogenesis and survival. However, most of these drugs have limitations; either metastatic ccRCC develops resistance to these agents, or despite blocking receptors, tumour cells use alternate signalling pathways. This review compiles the state of knowledge about the PI3K/AKT signalling pathway confined to ccRCC and its cross-talks with VHL/HIF pathway.

Carbonic anhydrase IX as a specific biomarker for clear cell renal cell carcinoma: comparative study of Western blot and immunohistochemistry and implications for diagnosis

2012 ◽  
Vol 46 (5) ◽  
pp. 358-364 ◽  
Author(s):  
José M. Giménez-Bachs ◽  
Antonio S. Salinas-Sánchez ◽  
Leticia Serrano-Oviedo ◽  
Syong H. Nam-Cha ◽  
Antonio Rubio-Del Campo ◽  
...  
Keyword(s):  
Renal Cell Carcinoma ◽  
Carbonic Anhydrase ◽  
Comparative Study ◽  
Western Blot ◽  
Cell Carcinoma ◽  
Renal Cell ◽  
Clear Cell ◽  
Carbonic Anhydrase Ix ◽  
Cell Renal Cell Carcinoma
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