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Renal cancer
Published in Anju Sahdev, Sarah J. Vinnicombe, Husband & Reznek's Imaging in Oncology, 2020
Conrad von Stempel, Lee Alexander Grant, Miles Walkden, Navin Ramachandran
Papillary RCC is the second most common subtype after clear cell RCC, accounting for up to 20% of RCC. Both papillary and chromophobe RCC tend to present with lower grade tumours and to be less likely to metastasize after treatment compared with clear cell RCC (34). Calcification is also more common in papillary and chromophobe RCC (in approximately one-third of cases) compared with clear cell RCC (11% demonstrate calcification) (67). Papillary RCC is a heterogeneous group of tumours with two distinct genetic and clinical subtypes, with some patients presenting with fairly indolent multifocal lesions and others with solitary aggressive tumours. Type 1 tumours are associated with hereditary papillary renal cell cancer syndrome and are often multifocal and bilateral. Non-hereditary type 1 papillary tumours are associated with the same proto-oncogene somatic mutations (MET) as in hereditary papillary renal cell carcinoma syndrome (68). Type 2 tumours are more likely to be solitary heterogeneous lesions. A more aggressive phenotype of type 2 papillary RCC is seen in patients with hereditary leiomyomatosis and renal-cell cancer syndromes (69).
Renal Cancer
Published in Manit Arya, Taimur T. Shah, Jas S. Kalsi, Herman S. Fernando, Iqbal S. Shergill, Asif Muneer, Hashim U. Ahmed, MCQs for the FRCS(Urol) and Postgraduate Urology Examinations, 2020
Nilay Patel (deceased), Vinodh Murali, David Cranston
The MOST common form of RCC in patients in end-stage renal disease and in patients on dialysis is:Clear cell RCCPapillary RCCChromophobe RCCCollecting duct RCCTubulocystic RCC
Hereditary Leiomyomatosis and Renal Cell Cancer
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Renal cell cancer (RCC) arises from the cells in the parenchyma of the kidneys, and consists of several histological types (e.g., clear cell RCC, papillary RCC, chromophobe RCC). Of these, papillary RCC is a peripheral lesion with necrosis, hemorrhage, and calcification, and shows tumor cells organized in a spindle-shaped pattern and possible areas of internal hemorrhage and cystic alterations. Papillary RCC can be further distinguished into two subtypes, with subtype 1 (basophilic, 73% of papillary RCC cases) being low grade and consisting of papillae lined by a single layer of small cells with scanty clear-to-basophilic cytoplasm and hyperchromatic nuclei, and subtype 2 (eosinophilic, 27% of papillary RCC cases) being high grade, and consisting of papillae lined by cells with abundant granular eosinophilic cytoplasm and prominent nucleoli, which is commonly found in HLRCC (Figure 31.3b) [8].
Molecular analysis and favorable clinical outcomes in real-world patients with metastatic renal cell carcinoma
Published in Acta Oncologica, 2022
Frede Donskov, Cathy Anne Pinto, Raluca Predoiu, Claire Fox, Jeanette Baehr Georgsen, Katrine Skaarup, Mehmet Burcu, Rodolfo Perini, Torben Steiniche
The comprehensive molecular characterization of clear cell renal cell carcinoma (ccRCC) and non-clear cell RCC (nccRCC) by the Cancer Genome Atlas (TCGA) Research Network has revealed the complexity of cancer [1–3]. For ccRCC, genetic changes were underpinned with the identification of 19 significantly mutated genes. Since then, Motzer identified seven molecular subtypes in the IMmotion 151 cohort [4,5] and a 26-gene signature in the JAVELIN Renal 101 cohort with differential outcomes to vascular endothelial growth factor (VEGF) blockade alone or in combination with programmed death-ligand 1 (PD-L1) inhibition [6], and McDermott identified a significant association of an 18 gene T-cell-inflamed gene expression profile (TcellinfGEP) in Keynote 427 with improved response to pembrolizumab monotherapy [7]. Analysis of biomarkers in nccRCC have been limited given the paucity of clinical trials for this disease. Comprehensive molecular characterization of primary papillary RCC revealed two types described by specific gene alterations [2]. An integrated comparative genomic analysis of ccRCC, papillary and chromophobe RCC confirmed a nearly universal upregulation of immune cell gene signatures for ccRCC compared with nccRCC [3]. Although there continues to be advancements in the understanding of disease biology, there is no single predictive biomarker to date that has been approved to select metastatic RCC patients for either immuno-oncology (I-O) or targeted therapies.
Investigational MET inhibitors to treat Renal cell carcinoma
Published in Expert Opinion on Investigational Drugs, 2019
Lakshminarayanan Nandagopal, Guru P. Sonpavde, Neeraj Agarwal
Papillary RCC (pRCC) is a distinct disease entity in RCC and is the most common subtype after clear cell RCC. It accounts for 15–20% of RCC and consists of two distinct clinical and molecular subtypes, with varied clinical features and outcomes. Type 1 is often multifocal, presents at earlier stage and has a more favorable prognosis. While the majority are sporadic (80%), activating germline mutations in MET occur in 10–20% of cases [28]. Hereditary pRCC is autosomal dominant and characterized by multifocal, slow growing lesions [29]. It is highly penetrant, with 90% carriers developing pRCC by the 8th decade [30]. The HPRC gene is located in the long arm of chromosome 7 and mutations lead to constitutive activation of the tyrosine kinase domain of MET [30]. The sporadic type 1 pRCC is characterized by alterations in the MET pathway in 80% of cases (mutations, splice variants, fusions) with the vast majority having duplication of chromosome 7 [31]. In another study of patients with pRCC with more advanced disease, a higher frequency of MET alterations and mutations were identified [32]. Thus, the MET signaling pathway plays a significant pathogenic role in both hereditary and sporadic subtypes of type 1 pRCC.
Biomarkers of aggressiveness in genitourinary tumors with emphasis on kidney, bladder, and prostate cancer
Published in Expert Review of Molecular Diagnostics, 2018
Alessia Cimadamore, Silvia Gasparrini, Matteo Santoni, Liang Cheng, Antonio Lopez-Beltran, Nicola Battelli, Francesco Massari, Francesca Giunchi, Michelangelo Fiorentino, Marina Scarpelli, Rodolfo Montironi
In ccRCC, apart from VHL alterations and the pathways responding to hypoxia, the TCGA analyses have demonstrated that aggressive tumors display a peculiar metabolic shift from the TCA cycle toward the pentose phosphate pathway and the glutamine transporter system [79]. Four main molecular subtypes of papillary RCC have been discovered. Type I pRCC tumors are closely related to the MET alteration in sporadic and hereditary tumors. By contrast, type II pRCC comprises at least three molecular subtypes associated with alterations in metabolic genes or mutations of CDKN2 or a high rate of CpG island methylation. The latter two molecular types are characterized by distinct clinical cancer aggressiveness [80].