"Clear cell renal cell carcinoma accounts for approximately 70–75% of renal cell carcinomas 11. There is a male predominance (roughly 2:1). The median age at diagnosis of sporadic ccRCC is in the early 60s. Hereditary cases (most commonly associated with von Hippel‑Lindau disease) present at a younger age, often in the 30s–40s and are frequently multifocal and bilateral 1,11."
"Clear cell renal cell carcinoma accounts for approximately 70–75% of renal cell carcinomas 11. There is a male predominance (roughly 2:1). The median age at diagnosis of sporadic ccRCC is in the early 60s. Hereditary cases (most commonly associated with von Hippel‑Lindau disease) present at a younger age, often in the 30s–40s and are frequently multifocal and bilateral 1,11."
"T1: typically heterogeneous, often isointense to slightly hypointense compared with cortex; intratumoral haemorrhage or proteinaceous content may increase signal"
"T2: usually hyperintense compared with the renal cortex, in contrast to the more commonly T2‑hypointense papillary RCC"
"in- and opposed-phase imaging: many ccRCCs demonstrate microscopic fat with focal or diffuse non‑curvilinear signal loss on opposed‑phase images; this is seen in a substantial proportion (around half in some series) but should not be mistaken for macroscopic fat of angiomyolipoma 6,7"
"Compared with papillary and chromophobe RCC, ccRCC has historically been more likely to present with locally advanced or metastatic disease, although stage migration has reduced this difference in contemporary series 11,12."
"Common to both sporadic and familial forms is the loss of sequences on the short arm of chromosome 3 in 98% of tumours, usually by deletion or unbalanced translocation resulting in loss of 3p12 to 3p26. The second allele often shows somatic mutation or epigenetic inactivation through hypermethylation. Interestingly, this region contains the sequence for the von Hippel-Lindau (VHL) protein, a component of an E3 ubiquitin ligase complex that directly targets HIF-1α for proteasomal degradation 1. The ubiquitin ligase complex normally identifies and tags proteins for destruction. Of particular relevance to clear cell carcinoma is ubiquitin-mediated degradation of hypoxia-inducible factor 1 (HIF-1), which is a pro-angiogenic factor normally expressed in hypoxic environments. As such, the loss of the VHL allele results in increased levels of HIF-1 and the resulting increase in pro-angiogenic factors such as VEGF, PDGF, TGF-α and TGF-β, leading to cellular dysplasia and, ultimately, neoplasia."
"Common to both sporadic and familial forms is the loss of sequences on the short arm of chromosome 3 in 98% of tumours, usually by deletion or unbalanced translocation resulting in loss of 3p12 to 3p26. The second allele often shows somatic mutation or epigenetic inactivation through hypermethylation. Interestingly, this region contains the sequence for the von Hippel-Lindau (VHL) protein, a component of an E3 ubiquitin ligase complex that directly targets HIF-1α for proteasomal degradation 1. The ubiquitin ligase complex normally identifies and tags proteins for destruction. Of particular relevance to clear cell carcinoma is ubiquitin-mediated degradation of hypoxia-inducible factor 1 (HIF-1), which is a pro-angiogenic factor normally expressed in hypoxic environments. As such, the loss of the VHL allele results in increased levels of HIF-1 and the resulting increase in pro-angiogenic factors such as VEGF, PDGF, TGF-α and TGF-β, leading to cellular dysplasia and, ultimately, neoplasia."
"Common to both sporadic and familial forms is the loss of sequences on the short arm of chromosome 3 in 98% of tumours, usually by deletion or unbalanced translocation resulting in loss of 3p12 to 3p26. The second allele often shows somatic mutation or epigenetic inactivation through hypermethylation. Interestingly, this region contains the sequence for the von Hippel-Lindau (VHL) protein, a component of an E3 ubiquitin ligase complex that directly targets HIF-1α for proteasomal degradation 1. The ubiquitin ligase complex normally identifies and tags proteins for destruction. Of particular relevance to clear cell carcinoma is ubiquitin-mediated degradation of hypoxia-inducible factor 1 (HIF-1), which is a pro-angiogenic factor normally expressed in hypoxic environments. As such, the loss of the VHL allele results in increased levels of HIF-1 and the resulting increase in pro-angiogenic factors such as VEGF, PDGF, TGF-α and TGF-β, leading to cellular dysplasia and, ultimately, neoplasia."
"Common to both sporadic and familial forms is the loss of sequences on the short arm of chromosome 3 in 98% of tumours, usually by deletion or unbalanced translocation resulting in loss of 3p12 to 3p26. The second allele often shows somatic mutation or epigenetic inactivation through hypermethylation. Interestingly, this region contains the sequence for the von Hippel-Lindau (VHL) protein, a component of an E3 ubiquitin ligase complex that directly targets HIF-1α for proteasomal degradation 1. The ubiquitin ligase complex normally identifies and tags proteins for destruction. Of particular relevance to clear cell carcinoma is ubiquitin-mediated degradation of hypoxia-inducible factor 1 (HIF-1), which is a pro-angiogenic factor normally expressed in hypoxic environments. As such, the loss of the VHL allele results in increased levels of HIF-1 and the resulting increase in pro-angiogenic factors such as VEGF, PDGF, TGF-α and TGF-β, leading to cellular dysplasia and, ultimately, neoplasia."
"Common to both sporadic and familial forms is the loss of sequences on the short arm of chromosome 3 in 98% of tumours, usually by deletion or unbalanced translocation resulting in loss of 3p12 to 3p26. The second allele often shows somatic mutation or epigenetic inactivation through hypermethylation. Interestingly, this region contains the sequence for the von Hippel-Lindau (VHL) protein, a component of an E3 ubiquitin ligase complex that directly targets HIF-1α for proteasomal degradation 1. The ubiquitin ligase complex normally identifies and tags proteins for destruction. Of particular relevance to clear cell carcinoma is ubiquitin-mediated degradation of hypoxia-inducible factor 1 (HIF-1), which is a pro-angiogenic factor normally expressed in hypoxic environments. As such, the loss of the VHL allele results in increased levels of HIF-1 and the resulting increase in pro-angiogenic factors such as VEGF, PDGF, TGF-α and TGF-β, leading to cellular dysplasia and, ultimately, neoplasia."
"Clear cell renal cell carcinoma often appears as an exophytic, well‑defined mass, but can also be infiltrative or centred in the renal sinus. The tumour typically shows strong heterogeneous enhancement on the corticomedullary and nephrographic phases, reflecting its rich vascularity; it usually enhances more than papillary RCC and often more than the renal cortex 4,5,12."
"T2: usually hyperintense compared with the renal cortex, in contrast to the more commonly T2‑hypointense papillary RCC"
"RENAL nephrometry scoring system"
"Common to both sporadic and familial forms is the loss of sequences on the short arm of chromosome 3 in 98% of tumours, usually by deletion or unbalanced translocation resulting in loss of 3p12 to 3p26. The second allele often shows somatic mutation or epigenetic inactivation through hypermethylation. Interestingly, this region contains the sequence for the von Hippel-Lindau (VHL) protein, a component of an E3 ubiquitin ligase complex that directly targets HIF-1α for proteasomal degradation 1. The ubiquitin ligase complex normally identifies and tags proteins for destruction. Of particular relevance to clear cell carcinoma is ubiquitin-mediated degradation of hypoxia-inducible factor 1 (HIF-1), which is a pro-angiogenic factor normally expressed in hypoxic environments. As such, the loss of the VHL allele results in increased levels of HIF-1 and the resulting increase in pro-angiogenic factors such as VEGF, PDGF, TGF-α and TGF-β, leading to cellular dysplasia and, ultimately, neoplasia."
Expected headings
"Radiographic appearance"
"Ultrasound"
"CT"
"MRI"
"Clear cell renal cell carcinoma accounts for approximately 70–75% of renal cell carcinomas 11. There is a male predominance (roughly 2:1). The median age at diagnosis of sporadic ccRCC is in the early 60s. Hereditary cases (most commonly associated with von Hippel‑Lindau disease) present at a younger age, often in the 30s–40s and are frequently multifocal and bilateral 1,11."
"Widespread use of cross‑sectional imaging means that many ccRCCs are now detected incidentally during imaging for unrelated indications 12. Symptomatic presentations include flank pain, haematuria and a palpable mass; paraneoplastic features such as anaemia, polycythaemia, hypercalcaemia or weight loss may occur 11."
"Widespread use of cross‑sectional imaging means that many ccRCCs are now detected incidentally during imaging for unrelated indications 12. Symptomatic presentations include flank pain, haematuria and a palpable mass; paraneoplastic features such as anaemia, polycythaemia, hypercalcaemia or weight loss may occur 11."
"Widespread use of cross‑sectional imaging means that many ccRCCs are now detected incidentally during imaging for unrelated indications 12. Symptomatic presentations include flank pain, haematuria and a palpable mass; paraneoplastic features such as anaemia, polycythaemia, hypercalcaemia or weight loss may occur 11."
"Clear cell renal cell carcinoma arises from the epithelium of the proximal tubule 1. Clear cell carcinoma is sporadic in over 95% of cases; in the 5% of familial cases, most are seen in von Hippel-Lindau disease 1."
"Clear cell renal cell carcinoma often appears as an exophytic, well‑defined mass, but can also be infiltrative or centred in the renal sinus. The tumour typically shows strong heterogeneous enhancement on the corticomedullary and nephrographic phases, reflecting its rich vascularity; it usually enhances more than papillary RCC and often more than the renal cortex 4,5,12."
"T1: typically heterogeneous, often isointense to slightly hypointense compared with cortex; intratumoral haemorrhage or proteinaceous content may increase signal"
"in- and opposed-phase imaging: many ccRCCs demonstrate microscopic fat with focal or diffuse non‑curvilinear signal loss on opposed‑phase images; this is seen in a substantial proportion (around half in some series) but should not be mistaken for macroscopic fat of angiomyolipoma 6,7"
"Common to both sporadic and familial forms is the loss of sequences on the short arm of chromosome 3 in 98% of tumours, usually by deletion or unbalanced translocation resulting in loss of 3p12 to 3p26. The second allele often shows somatic mutation or epigenetic inactivation through hypermethylation. Interestingly, this region contains the sequence for the von Hippel-Lindau (VHL) protein, a component of an E3 ubiquitin ligase complex that directly targets HIF-1α for proteasomal degradation 1. The ubiquitin ligase complex normally identifies and tags proteins for destruction. Of particular relevance to clear cell carcinoma is ubiquitin-mediated degradation of hypoxia-inducible factor 1 (HIF-1), which is a pro-angiogenic factor normally expressed in hypoxic environments. As such, the loss of the VHL allele results in increased levels of HIF-1 and the resulting increase in pro-angiogenic factors such as VEGF, PDGF, TGF-α and TGF-β, leading to cellular dysplasia and, ultimately, neoplasia."