gms | German Medical Science

56. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie e. V. (DGNC)
3èmes journées françaises de Neurochirurgie (SFNC)

Deutsche Gesellschaft für Neurochirurgie e. V.
Société Française de Neurochirurgie

07. bis 11.05.2005, Strasbourg

Treatment of hemangioblastomas in adolescent patients with von Hippel-Lindau disease

Chirugische Therapie von Hemangioblastomen bei jugendlichen Patienten mit von Hippel Lindau Erkrankung

Meeting Abstract

  • corresponding author V. I. Vougioukas - Neurochirurgische Klinik, Universitätsklinikum Freiburg
  • S. Gläsker - Neurochirurgische Klinik, Universitätsklinikum Freiburg
  • V. Van Velthoven - Neurochirurgische Klinik, Universitätsklinikum Freiburg

Deutsche Gesellschaft für Neurochirurgie. Société Française de Neurochirurgie. 56. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie e.V. (DGNC), 3èmes journées françaises de Neurochirurgie (SFNC). Strasbourg, 07.-11.05.2005. Düsseldorf, Köln: German Medical Science; 2005. Doc09.05.-15.06

The electronic version of this article is the complete one and can be found online at:

Published: May 4, 2005

© 2005 Vougioukas et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.




Hemangioblastomas are highly vascular tumors that occur as sporadic lesions or components of the autosomal dominant von Hippel-Lindau (VHL) disease. Aim of the study was to define the role of preoperative genetic screening, timing of surgery and surgical technique in the treatment of pediatric VHL patients.


We retrospectively analyzed clinical and radiological data of 14 pediatric patients with VHL disease and central nervous system (CNS) hemangioblastomas that were surgically treated in our clinic between 1974 and 2004 (mean age: 15.1 years). Eight patients were operated on cerebellar, 2 on brainstem and 4 on intramedullary hemangioblastomas. Functional outcomes were assessed. All patients and their families were screened for mutations and deletions of the VHL gene.


None of the preoperatively symptomatic patients developed a new neurological deficit. Two asymptomatic patients were surgically treated because of a radiologically validated tumor size progression. Peritumoral edema in the preoperative images was correlated with significantly higher surgical morbidity.


Microsurgical removal of hemangioblastomas in the pediatric age group can be performed with low morbidity and excellent outcome. According to our data asymptomatic hemagioblastomas with proven radiological progression should be removed in order to avoid irreversible neurological deficits. Genetic screening of VHL patients and their families is essential for the development of an efficient therapeutic strategy.