Kidney cancer. Principles and practice. Second edition. Primo N. Lara, Jr. Eric Jonasch (Editors). Springer International Publishing (2015)

Hereditary kidney cancer accounts for 5–8% of the total number of RCC diagnosed, but its clinical significance cannot be overstated. Clinicians should have a heightened index of suspicion when a patient presents with bilateral, multifocal lesions at a young age and has a strong family history of RCC, or if he/she shows the characteristic extrarenal manifestations. Management of such patients follows three principal tenets: early diagnosis, rigorous surveillance, and interventions that maximize renal function. Genetic testing and management require a multidisciplinary team including a clinical geneticist. Ascertainment of the genetic defect not only dictates the course of treatment for the patient but also has bearing on the health management of the living relatives. Finally, surgical resection should be undertaken with the considerations of sound oncologic extirpation weighed against the preservation of renal function. Despite our advancements in genetics, surgical techniques, and pharmacotherapies, it is apparent that much work remains to fully delineate this group of heritable cancers.

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