• Pascalle Mossel, van der Horst-SchriversAnouk N AANADepartment of Endocrinology, HPC, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands., Maran J W Olderode-Berends, GroenRob J MRJMDepartment of Neurosurgery, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands., Eelco W Hoving, Auke P A Appelman, and Thera P Links.
• Department of Nuclear Medicine and Molecular Imaging, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
• World Neurosurg. 2022 Dec 1; 168: e67e75e67-e75.

ObjectiveHemangioblastomas in the central nervous system are the most common manifestation of von Hippel-Lindau (VHL) disease. Because the growth rate of hemangioblastomas is unpredictable, regular follow-up is mandatory, focusing on clinical symptoms and imaging of the central nervous system. However, clinical symptoms may be subtle and nonspecific, and data about the relationship between the radiologic findings and clinical symptoms are sparse. This study aims to evaluate if and how findings of magnetic resonance imaging (MRI) regarding spinal hemangioblastomas are associated with symptoms of VHL disease, with special attention to peritumoral edema and spinal cysts.MethodsSerial spinal MRI scans of 43 genetically or clinically established VHL patients with at least 2 years of follow-up were reevaluated to examine the volume, growth rate, and location of spinal hemangioblastomas and the presence, size, and growth rate of peritumoral edema and cysts. Findings were compared with clinical symptoms using the Fisher exact test.ResultsWe observed a total of 77 spinal hemangioblastomas in 28 patients. Eight of the 28 patients showed peritumoral edema and spinal cysts, and 1 patient showed peritumoral edema without cyst formation; 6 of these 9 patients showed clinical symptoms. Both peritumoral edema and spinal cysts were associated with clinical symptoms (P = 0.023 and P = 0.011, respectively).ConclusionsThe presence of peritumoral edema and/or spinal cysts shown on MRI in VHL patients with spinal hemangioblastomas is associated with symptoms in more than half of the patients and may alert the clinician to intensify clinical and radiologic surveillance.Copyright © 2022 The Author(s). Published by Elsevier Inc. All rights reserved.

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