Objectives: Hereditary renal cancer syndromes have been described and have illuminated novel methods to treat sporadic renal cell carcinoma. In this work, we aimed to review the genetic basis, molecular pathology and clinical manifestations of hereditary syndromes, as well as outline principles of surgical management and use of targeted therapy.
Methods: We performed a comprehensive review of selected peer-reviewed publications regarding hereditary renal cancer syndromes, their genetic basis, and recommendations for surgical management.
Results: The major syndromes contributing to hereditary renal cell carcinoma are discussed along with relevant literature guiding their management. The evolving surgical and molecular treatments are discussed.
Conclusions: Identification of genetic basis of hereditary carcinomas provides opportunity for targeted therapy of metastatic sporadic renal cell carcinoma. Appropriate and timely surgical management of hereditary renal cancers decreases the possibility of development of metastatic disease, and allows for preservation of renal function despite the need for repeat surgical interventions.