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Surgical Management of Hereditary Kidney Cancer Syndromes

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Integrating Multidisciplinary Treatment for Advanced Renal Cell Carcinoma

Abstract

Hereditary kidney cancer accounts for 5–8% of all diagnosed kidney cancer. Afflicted patients are often at risk for developing bilateral, multifocal tumors throughout their lifetime. Over a dozen tumor-predisposing genes have been identified, each resulting in a unique clinical syndrome that, in almost all instances, also has extrarenal manifestations. In this chapter, we discuss the common hereditary renal cancers, for each describing their underlying genetic alteration; clinical phenotype; and management in the localized setting. By understanding the biological footprint of unique genetic signatures, specific surgical decision-making and, in some cases, medical therapy has been employed based on a patient’s germline alteration. While incomplete, the precision-based approach towards managing hereditary kidney cancer can serve as a model with which to apply to sporadic renal cell carcinoma.

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  1. Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA

    Nikhil Gopal, Bradley Webster, Maria Antony & Mark W. Ball

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    Eric A. Singer

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Gopal, N., Webster, B., Antony, M., Ball, M.W. (2023). Surgical Management of Hereditary Kidney Cancer Syndromes. In: McKay, R.R., Singer, E.A. (eds) Integrating Multidisciplinary Treatment for Advanced Renal Cell Carcinoma. Springer, Cham. https://doi.org/10.1007/978-3-031-40901-1_6

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