NCCN Guidelines Insights: Kidney Cancer, Version 1.2021

Featured Updates to the NCCN Guidelines

Authors:
Robert J. Motzer Memorial Sloan Kettering Cancer Center;

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Eric Jonasch The University of Texas MD Anderson Cancer Center;

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Shawna Boyle Fred & Pamela Buffett Cancer Center;

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Maria I. Carlo Memorial Sloan Kettering Cancer Center;

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Brandon Manley Moffitt Cancer Center;

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Neeraj Agarwal Huntsman Cancer Institute at the University of Utah;

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Ajjai Alva University of Michigan Rogel Cancer Center;

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Katy Beckermann Vanderbilt-Ingram Cancer Center;

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Toni K. Choueiri Dana-Farber/Brigham and Women’s Cancer Center;

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Brian A. Costello Mayo Clinic Cancer Center;

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Ithaar H. Derweesh UC San Diego Moores Cancer Center;

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Arpita Desai UCSF Helen Diller Family Comprehensive Cancer Center;

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Saby George Roswell Park Comprehensive Cancer Center;

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John L. Gore Fred Hutchinson Cancer Research Center/Seattle Cancer Care Alliance;

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Naomi Haas Abramson Cancer Center at the University of Pennsylvania;

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Steven L. Hancock Stanford Cancer Institute;

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Christos Kyriakopoulos University of Wisconsin Carbone Cancer Center;

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Elaine T. Lam University of Colorado Cancer Center;

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Clayton Lau City of Hope National Medical Center;

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Bryan Lewis Kidney Cancer Coalition;

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David C. Madoff Yale Cancer Center/Smilow Cancer Hospital;

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Brittany McCreery Fred Hutchinson Cancer Research Center/Seattle Cancer Care Alliance;

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M. Dror Michaelson Massachusetts General Hospital Cancer Center;

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Amir Mortazavi The Ohio State University Comprehensive Cancer Center - James Cancer Hospital and Solove Research Institute;

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Lakshminarayanan Nandagopal O'Neal Comprehensive Cancer Center at UAB;

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Phillip M. Pierorazio The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins;

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Elizabeth R. Plimack Fox Chase Cancer Center;

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Lee Ponsky Case Comprehensive Cancer Center/ University Hospitals Seidman Cancer Center and Cleveland Clinic Taussig Cancer Institute;

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Sundhar Ramalingam Duke Cancer Institute;

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Brian Shuch UCLA Jonsson Comprehensive Cancer Center;

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Zachary L. Smith Siteman Cancer Center at Barnes-Jewish Hospital and Washington University School of Medicine;

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Bradley Somer St. Jude Children’s Research Hospital/The University of Tennessee Health Science Center;

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Jeffrey Sosman Robert H. Lurie Comprehensive Cancer Center of Northwestern University; and

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Mary A. Dwyer National Comprehensive Cancer Network.

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Angela D. Motter National Comprehensive Cancer Network.

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The NCCN Guidelines for Kidney Cancer provide multidisciplinary recommendations for diagnostic workup, staging, and treatment of patients with renal cell carcinoma (RCC). These NCCN Guidelines Insights focus on recent updates to the guidelines, including changes to certain systemic therapy recommendations for patients with relapsed or stage IV RCC. They also discuss the addition of a new section to the guidelines that identifies and describes the most common hereditary RCC syndromes and provides recommendations for genetic testing, surveillance, and/or treatment options for patients who are suspected or confirmed to have one of these syndromes.

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  • 3.

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    Lipworth L, Morgans AK, Edwards TL, et al.. Renal cell cancer histological subtype distribution differs by race and sex. BJU Int 2016;117:260265.

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    U.S. Food & Drug Administration. FDA approves pembrolizumab plus axitinib for advanced renal cell carcinoma. Accessed July 20, 2020. Available at: https://www.fda.gov/drugs/drug-approvals-and-databases/fda-approves-pembrolizumab-plus-axitinib-advanced-renal-cell-carcinoma

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    Rini BI, Plimack ER, Stus V, et al.. Pembrolizumab plus axitinib versus sunitinib for advanced renal-cell carcinoma. N Engl J Med 2019;380:11161127.

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    Lenvatinib. Prescribing information. Eisai; 2020. Accessed July 20, 2020. Available at: http://www.lenvima.com/pdfs/prescribing-information.pdf

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  • 12.

    U.S. Food & Drug Administration. FDA approves lenvatinib in combination with everolimus for advanced renal cell carcinoma. Accessed July 20, 2020. Available at: https://www.fda.gov/drugs/resources-information-approved-drugs/lenvatinib-combination-everolimus

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    Motzer RJ, Hutson TE, Glen H, et al.. Lenvatinib, everolimus, and the combination in patients with metastatic renal cell carcinoma: a randomised, phase 2, open-label, multicentre trial. Lancet Oncol 2015;16:14731482.

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    de Velasco G, McKay RR, Lin X, et al.. Comprehensive analysis of survival outcomes in non-clear cell renal cell carcinoma patients treated in clinical trials. Clin Genitourin Cancer 2017;15:652660.e651.

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    Hutson TE, Michaelson MD, Kuzel TM, et al.. A phase II study of lenvatinib plus everolimus in patients with advanced non-clear cell renal cell carcinoma (nccRCC) [abstract]. J Clin Oncol 2020;38(Suppl):Abstract 685.

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  • 16.

    Menko FH, Maher ER, Schmidt LS, et al.. Hereditary leiomyomatosis and renal cell cancer (HLRCC): renal cancer risk, surveillance and treatment. Fam Cancer 2014;13:637644.

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    Rednam SP, Erez A, Druker H, et al.. Von Hippel-Lindau and hereditary pheochromocytoma/paraganglioma syndromes: clinical features, genetics, and surveillance recommendations in childhood. Clin Cancer Res 2017;23:e6875.

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    Tufton N, Sahdev A, Akker SA. Radiological surveillance screening in asymptomatic succinate dehydrogenase mutation carriers. J Endocr Soc 2017;1:897907.

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    Eijkelenkamp K, Osinga TE, de Jong MM, et al.. Calculating the optimal surveillance for head and neck paraganglioma in SDHB-mutation carriers. Fam Cancer 2017;16:123130.

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    Menko FH, van Steensel MA, Giraud S, et al.. Birt-Hogg-Dubé syndrome: diagnosis and management. Lancet Oncol 2009;10:11991206.

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    Star P, Goodwin A, Kapoor R, et al.. Germline BAP1-positive patients: the dilemmas of cancer surveillance and a proposed interdisciplinary consensus monitoring strategy. Eur J Cancer 2018;92:4853.

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  • 23.

    Ornstein DK, Lubensky IA, Venzon D, et al.. Prevalence of microscopic tumors in normal appearing renal parenchyma of patients with hereditary papillary renal cancer. J Urol 2000;163:431433.

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    Pavlovich CP, Grubb RL III, Hurley K, et al.. Evaluation and management of renal tumors in the Birt-Hogg-Dubé syndrome. J Urol 2005;173:14821486.

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    Shuch B, Singer EA, Bratslavsky G. The surgical approach to multifocal renal cancers: hereditary syndromes, ipsilateral multifocality, and bilateral tumors. Urol Clin North Am 2012;39:133148.

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    Gill AJ, Hes O, Papathomas T, et al.. Succinate dehydrogenase (SDH)-deficient renal carcinoma: a morphologically distinct entity: a clinicopathologic series of 36 tumors from 27 patients. Am J Surg Pathol 2014;38:15881602.

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    Singer EA, Vourganti S, Lin KY, et al.. Outcomes of patients with surgically treated bilateral renal masses and a minimum of 10 years of followup. J Urol 2012;188:20842088.

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  • 28.

    Everolimus. Prescribing information. Novartis; 2020. Accessed July 20, 2020. Available at: https://www.novartis.us/sites/www.novartis.us/files/afinitor.pdf

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  • 29.

    Bissler JJ, Kingswood JC, Radzikowska E, et al.. Everolimus for angiomyolipoma associated with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis (EXIST-2): a multicentre, randomised, double-blind, placebo-controlled trial. Lancet 2013;381:817824.

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  • 30.

    Srinivasan R, Su D, Stamatakis L, et al.. Mechanism based targeted therapy for hereditary leiomyomatosis and renal cell cancer (HLRCC) and sporadic papillary renal cell carcinoma: interim results from a phase 2 study of bevacizumab and erlotinib [abstract]. Eur J Cancer 2014;50:Abstract 8.

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  • 31.

    Jonasch E, McCutcheon IE, Gombos DS, et al.. Pazopanib in patients with von Hippel-Lindau disease: a single-arm, single-centre, phase 2 trial. Lancet Oncol 2018;19:13511359.

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