Radiologic Characteristics of Spinal Hemangioblastomas in von Hippel Lindau Disease as Guidance in Clinical Interventions.

Autor: Mossel P; Department of Nuclear Medicine and Molecular Imaging, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands., van der Horst-Schrivers ANA; Department of Endocrinology, HPC, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands., Olderode-Berends MJW; Department of Genetics, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands., Groen RJM; Department of Neurosurgery, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands., Hoving EW; Department of Neurosurgery, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands; Clinical Director Neuro-Oncology in the Princess Maxima Center for Pediatric Oncology, Utrecht, The Netherlands., Appelman APA; Department of Radiology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands., Links TP; Department of Endocrinology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. Electronic address: t.p.links@umcg.nl.
Jazyk: angličtina
Zdroj: World neurosurgery [World Neurosurg] 2022 Dec; Vol. 168, pp. e67-e75. Date of Electronic Publication: 2022 Sep 17.
DOI: 10.1016/j.wneu.2022.09.011
Abstrakt: Objective: Hemangioblastomas 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.
Methods: Serial 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.
Results: We 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).
Conclusions: The 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.)
Databáze: MEDLINE
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