Evidence-Based Guidelines for the Screening, Surveillance, and Management of VHL Manifestations

Patients with VHL disease develop tumors affecting various organ systems and anatomical locations starting at an early age. Effective management of this disease involves detecting and intervening on these tumors at appropriate timepoints before the development of symptoms or progression to metastatic disease. Screening and surveillance guidelines based on our understanding of the natural history of VHL disease have been developed to guide management of these complex tumors. The goal is to reduce tumor-related morbidity and mortality while preserving organ functions. This chapter summarizes the screening and surveillance guidelines put forth by various international societies as well as the general management strategies for various VHL lesions. In short, patients with VHL should enter screening and surveillance programs—ideally at institutions with multidisciplinary clinics specializing in VHL disease management—at early age and should undergo annual or biennial follow-up to track their disease progression and determine the appropriate time for intervention if needed.