Stereotactic Radiosurgery for Central Nervous System Hemangioblastoma in Von Hippel Lindau Disease

Introduction: Von Hippel Lindau disease is an autosomal dominant genetic disorder characterized by the presence of multifocal tumors that are generally benign. 80% of patients with this condition develop hemangioblastomas (HB) that are predominantly multifocal (90%), with a primary location at the level of the central nervous system (CNS), and are asymptomatic for long periods of time despite their progressive nature.

Methods: A systematic search was performed in PubMed, Cochrane Library, and Scopus. It focused on studies related to hemangioblastoma of the nervous system, VHL, and its treatment with stereotactic radiosurgery in terms of safety and efficacy.

Results: In the present study, a comprehensive analysis was performed which involved the study of 180 patients from different regions with hemangioblastomas associated with Von Hippel-Lindau disease with a mean age of 37 years who had a surgical approach using SRS. Thus, a survival rate of 93.5% was observed at 3 years, which subsequently decreased to 86% at 5 years and 70% at 10 years. Stereotactic radiosurgery (SRS) is a noninvasive adjuvant or salvage treatment option that offers satisfactory tumor control for the regression or control of hemangioblastomas in patients with VHL.

Conclusion : The present study reinforces the use of stereotactic radiosurgery as an effective and less invasive therapeutic modality for the management of hemangioblastomas associated with Von Hippel-Lindau disease. Highlighting the unique characteristics of the modality, allowing precise targeting and minimizing complications.