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

Pascalle Mossel; Anouk N A van der Horst-Schrivers; Maran J W Olderode-Berends; Rob J M Groen; Eelco W Hoving; Auke P A Appelman; Thera P Links

doi:10.1016/j.wneu.2022.09.011

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

World Neurosurg. 2022 Dec:168:e67-e75. doi: 10.1016/j.wneu.2022.09.011. Epub 2022 Sep 17.

Authors

Pascalle Mossel¹, Anouk N A van der Horst-Schrivers², Maran J W Olderode-Berends³, Rob J M Groen⁴, Eelco W Hoving⁵, Auke P A Appelman⁶, Thera P Links⁷

Affiliations

¹ Department of Nuclear Medicine and Molecular Imaging, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
² Department of Endocrinology, HPC, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
³ Department of Genetics, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
⁴ Department of Neurosurgery, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
⁵ 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.
⁶ Department of Radiology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
⁷ Department of Endocrinology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. Electronic address: t.p.links@umcg.nl.

PMID: 36126890
DOI: 10.1016/j.wneu.2022.09.011

Abstract

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.

Keywords: Peritumoral edema; Spinal cyst; Spinal hemangioblastoma; Von Hippel-Lindau disease.

MeSH terms

Cysts* / complications
Edema
Follow-Up Studies
Hemangioblastoma* / complications
Hemangioblastoma* / diagnostic imaging
Hemangioblastoma* / surgery
Humans
Spinal Cord Neoplasms* / diagnosis
von Hippel-Lindau Disease* / complications
von Hippel-Lindau Disease* / diagnostic imaging