An evaluation of the Danish national clinical guidelines for von Hippel-Lindau (VHL)

Von Hippel-Lindau (VHL) is a rare hereditary and potentially fatal cancer syndrome. Because of its unpredictable manifestations in various organ systems, surveillance is not linked to a single department and may therefore be incomplete. Denmark published national guidelines for the surveillance of patients with manifest and possible VHL in 2005 and was one of the first countries to do so. The present study is the first of its kind; patients with suspected and manifest VHL were followed at a single institution according to the national guidelines. The purpose was to evaluate (1) to what extent the guidelines were being followed and (2) what findings were disclosed.