RCC is diagnosed by a combination of imaging and pathology to confirm malignancy, and to stage patients both clinically and pathologically.
TNM staging of RCC[7]Amin MB, Edge S, Green F, et al. AJCC cancer staging manual. 8th ed. (2017). Cham, Switzerland: Springer International; 2017.[8]Brierley JD, Gospodarowicz MK, Wittekind C, eds. Union for International Cancer Control. TNM classification of malignant tumors. 8th ed. Chichester: Wiley-Blackwell; 2017.
The TNM classification describes the extent of disease based on the following anatomic factors:
size and extent of the primary tumor (T)
regional lymph node involvement (N); and
presence or absence of distant metastases (M).
Staging
Early-stage RCC (stages 1 and 2) is defined as tumor confined to kidney without regional lymph node or distant metastasis.
Stage 3 tumors extend into major veins, or invade the adrenal gland or perinephric tissue, but do not invade beyond the Gerota fascia. There may be metastasis in a single regional lymph node, but no evidence of distant metastasis.
Stage 4 tumors extend beyond the Gerota fascia or have distant metastasis.
Prognostic criteria
Prognostic models have been developed to integrate these diagnostic findings into a schema that includes other patient factors, and that may better predict for survival or prognosis than classic staging; however, the utility of these models to date has largely been for stratifying patients into clinical trials.
The UCLA Integrated Staging System and SSIGN (Mayo) algorithms can be applied to patients with early localized disease, postnephrectomy.[74]Zisman A, Pantuck AJ, Dorey F, et al. Improved prognostication of renal cell carcinoma using an integrated staging system. J Clin Oncol. 2001 Mar 15;19(6):1649-57.
http://www.ncbi.nlm.nih.gov/pubmed/11250993?tool=bestpractice.com
[75]Frank I, Blute ML, Cheville JC, et al. An outcome prediction model for patients with clear cell renal cell carcinoma treated with radical nephrectomy based on tumor stage, size, grade, and necrosis: the SSIGN Score. J Urol. 2002 Dec;168(6):2395-400.
http://www.ncbi.nlm.nih.gov/pubmed/12441925?tool=bestpractice.com
The Memorial Sloan Kettering Cancer Center (MSKCC) model is one of the more widely used prognostic models in metastatic disease, and has been well validated.[60]Motzer RJ, Bacik J, Mazumdar M. Prognostic factors for survival of patients with stage IV renal cell carcinoma: Memorial Sloan-Kettering Cancer Center experience. Clin Cancer Res. 2004 Sep 15;10(18 Pt 2):6302S-3S.
http://clincancerres.aacrjournals.org/content/10/18/6302S.long
http://www.ncbi.nlm.nih.gov/pubmed/15448021?tool=bestpractice.com
Further prognostic criteria for patients with metastatic RCC treated with sunitinib, sorafenib, and bevacizumab have been elucidated, in addition to validation of some of the MSKCC criteria.[76]Heng DY, Xie W, Regan MM, et al. Prognostic factors for overall survival in patients with metastatic renal cell carcinoma treated with vascular endothelial growth factor-targeted agents: results from a large, multicenter study. J Clin Oncol. 2009 Dec 1;27(34):5794-9.
http://www.ncbi.nlm.nih.gov/pubmed/19826129?tool=bestpractice.com
This study found that hemoglobin less than the lower limit of normal, corrected calcium greater than the upper limit of normal (ULN), Karnofsky performance status less than 80%, time from diagnosis to treatment of less than 1 year, lactate dehydrogenase greater than 1.5 times the ULN, neutrophils greater than the ULN, and platelets greater than the ULN were independent adverse prognostic factors.[76]Heng DY, Xie W, Regan MM, et al. Prognostic factors for overall survival in patients with metastatic renal cell carcinoma treated with vascular endothelial growth factor-targeted agents: results from a large, multicenter study. J Clin Oncol. 2009 Dec 1;27(34):5794-9.
http://www.ncbi.nlm.nih.gov/pubmed/19826129?tool=bestpractice.com
The other most commonly used risk stratification score is the International Metastatic Renal Cell Carcinoma Database Consortium (IMDC) criteria, which includes neutrophilia and thrombocytosis.[77]Heng DY, Xie W, Regan MM, et al. External validation and comparison with other models of the
International Metastatic Renal-Cell Carcinoma Database Consortium prognostic model: a population-based study. Lancet Oncol. 2013 Feb;14(2):141-8.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4144042
http://www.ncbi.nlm.nih.gov/pubmed/23312463?tool=bestpractice.com
Predictors of poor survival are:[77]Heng DY, Xie W, Regan MM, et al. External validation and comparison with other models of the
International Metastatic Renal-Cell Carcinoma Database Consortium prognostic model: a population-based study. Lancet Oncol. 2013 Feb;14(2):141-8.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4144042
http://www.ncbi.nlm.nih.gov/pubmed/23312463?tool=bestpractice.com
Karnofsky performance status of less than 80%
Less than 1 year from diagnosis to treatment
Anemia (hemoglobin concentration less than the lower limit of normal)
Hypercalcemia (corrected calcium concentration greater than the ULN)
Neutrophilia (neutrophil count greater than the ULN)
Thrombocytosis (platelet count greater than the ULN).
Risk groups in the MSKCC and IMDC models are categorized as:[77]Heng DY, Xie W, Regan MM, et al. External validation and comparison with other models of the
International Metastatic Renal-Cell Carcinoma Database Consortium prognostic model: a population-based study. Lancet Oncol. 2013 Feb;14(2):141-8.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4144042
http://www.ncbi.nlm.nih.gov/pubmed/23312463?tool=bestpractice.com
A novel risk scoring system for patients with metastatic RCC treated with immune checkpoint inhibitors has been proposed, and includes monocyte‐to‐lymphocyte ratio, BMI, and number and sites of metastases at baseline; however, this is awaiting validation.[78]Martini DJ, Liu Y, Shabto JM, et al. Novel risk scoring system for patients with metastatic renal cell carcinoma treated with immune checkpoint inhibitors. Oncologist. 2020 Mar;25(3):e484-91.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7066702
http://www.ncbi.nlm.nih.gov/pubmed/32162798?tool=bestpractice.com
Molecular profiling and biomarkers
The effort to integrate molecular profiling and biomarkers into prognostic models continues, particularly for metastatic disease. Several molecular biomarkers are emerging as potential correlates of disease extent, prognosis, predictive markers of response to therapy, and even as potential therapeutic targets.[2]Escudier B, Porta C, Schmidinger M, et al. Renal cell carcinoma: ESMO clinical practice guidelines for diagnosis, treatment, and follow-up. Ann Oncol. 2019 May;30(5):706-20.
https://www.annalsofoncology.org/article/S0923-7534(19)31157-3/fulltext
http://www.ncbi.nlm.nih.gov/pubmed/30788497?tool=bestpractice.com
Current biomarkers of interest include plasma protein levels of vascular endothelial growth factor, hypoxia induced factor, tissue inhibitor of metalloproteinase 1; various genetic mutations such as in VHL, ras p21, pTEN loss, and chromosomal abnormalities such as loss of 9p; and tumor expression of cell death-related molecules (programmed death 1, programmed cell death 1 ligand 1, cytotoxic T-lymphocyte-associated protein-4).[79]Peña C, Lathia C, Shan M, et al. Biomarkers predicting outcome in patients with advanced renal cell carcinoma: results from sorafenib phase III Treatment Approaches in Renal Cancer Global Evaluation Trial. Clin Cancer Res. 2010 Oct 1;16(19):4853-63.
http://www.ncbi.nlm.nih.gov/pubmed/20651059?tool=bestpractice.com
[80]Calvo E, Porta C, Grünwald V, et al. The current and evolving landscape of first-line treatments for advanced renal cell carcinoma. Oncologist. 2019 Mar;24(3):338-48.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6519762
http://www.ncbi.nlm.nih.gov/pubmed/30158285?tool=bestpractice.com
[81]Rini BI, Battle D, Figlin RA, et al. The society for immunotherapy of cancer consensus statement on immunotherapy for the treatment of advanced renal cell carcinoma (RCC). J Immunother Cancer. 2019 Dec 20;7(1):354.
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6924043
http://www.ncbi.nlm.nih.gov/pubmed/31856918?tool=bestpractice.com