Article
Treatment of hemangioblastomas in adolescent patients with von Hippel-Lindau disease
Chirugische Therapie von Hemangioblastomen bei jugendlichen Patienten mit von Hippel Lindau Erkrankung
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Authors
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V. I. Vougioukas
- Neurochirurgische Klinik, Universitätsklinikum Freiburg
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S. Gläsker
- Neurochirurgische Klinik, Universitätsklinikum Freiburg
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V. Van Velthoven
- Neurochirurgische Klinik, Universitätsklinikum Freiburg
| Published: | May 4, 2005 |
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Outline
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Objective
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.
Methods
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.
Results
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.
Conclusions
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.
