Tuberous sclerosis complex and von Hippel–Lindau disease are distinct autosomal dominant tumor suppressor syndromes that are characterized by solid and cystic renal lesions. The products of the affected genes are linked in an intricate pathway that regulates cell growth, proliferation, and vascular supply. This Review examines the phenotypic overlap between the diseases in the context of their pathophysiologic similarities, outlines the optimal management of both conditions, and highlights promising future pharmacologic interventions.
- Brian J Siroky
- Maria F Czyzyk-Krzeska
- John J Bissler
