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Urol Clin North Am. 2017 May;44(2):155-167. doi: 10.1016/j.ucl.2016.12.002. Epub 2017 Mar 14.

Hereditary Kidney Cancer Syndromes and Surgical Management of the Small Renal Mass.

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Department of Urology, Yale School of Medicine, 789 Howard Avenue, New Haven, CT 06520, USA.
Department of Radiology, Yale School of Medicine, PO Box 208058, New Haven, CT 06520-8058, USA; Department of Urology, Yale School of Medicine, PO Box 208058, New Haven, CT 06520-8058, USA. Electronic address:


The management of patients with hereditary kidney cancers presents unique challenges to clinicians. In addition to an earlier age of onset compared with patients with sporadic kidney cancer, those with hereditary kidney cancer syndromes often present with bilateral and/or multifocal renal tumors and are at risk for multiple de novo lesions. This population of patients may also present with extrarenal manifestations, which adds an additional layer of complexity. Physicians who manage these patients should be familiar with the underlying clinical characteristics of each hereditary kidney cancer syndrome and the suggested surgical approaches and recommendations of genetic testing for at-risk individuals.


Germline alterations; Hereditary syndrome; Multifocal kidney cancer; Nephron-sparing surgery; Renal cell carcinoma

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