Intradural cauda equina hemangioblastomas: Surgical management and long-term outcomes at a von Hippel-Lindau referral center.
- 1Department of Neurosurgery, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Germany.
- 2Department of Neurosurgery and Spine Surgery, Klinikum Main-Spessart, Lohr am Main, Germany.
- 3Renal Division, Department of Medicine, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Germany.
Abstract
Introduction
Spinal hemangioblastomas (sHBLs) are rare vascular tumors occurring sporadically or in association with von Hippel-Lindau (VHL) disease. Intradural cauda equina sHBLs are uncommon and understudied.Research question
We aimed to describe their surgical treatment and outcomes at a VHL referral center.Methods
We retrospectively analyzed a prospectively maintained, surgically ascertained VHL/HBL registry of 237 patients who underwent 565 procedures for 804 resected HBLs between January 1997 and July 2025, with follow-up through February 2026. Functional status was assessed using McCormick grade.Results
Intradural cauda equina sHBLs were identified in 32/237 registry patients (13.5%). Eight patients (25%) underwent eight procedures involving ten tumors; the remaining 24 patients had no resection. Reasons for resection were symptoms and/or radiological progression (n = 7), concomitant removal during surgery for another lesion (n = 2), and for diagnostics due to initial presentation (n = 1). All ten tumors were completely resected. At first follow-up, McCormick grade was unchanged in all eight patients, and radicular pain improved in all six symptomatic patients. Median imaging follow-up was 67 months (range, 11-260), with no recurrence was identified. One postoperative cerebrospinal fluid fistula resolved after lumbar drainage. One patient experienced a clinical deterioration after 200 months due to VHL disease burden.Discussion and conclusion
Cauda equina sHBLs were infrequently resected in this selected registry. At our center, management is individualized within a structured surveillance program. Surgical decisions are guided by symptoms, radiological progression, lesion location, and clinical context, with favorable outcome. Long-term surveillance at an experienced VHL center remains essential.
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BibTeX
@article{doriamedina2026intradural,
title = {{Intradural cauda equina hemangioblastomas: Surgical management and long-term outcomes at a von Hippel-Lindau referral center.}},
author = {Roberto Doria-Medina and Nicolas Neidert and Marc Hohenhaus and Christine Julia Gizaw and Kilian Rhein and Athina Ganner and Romina Hohenhaus and Jürgen Beck and Jan-Helge Klingler},
journal = {Brain \& spine},
year = {2026},
month = oct,
doi = {10.1016/j.bas.2026.107479},
url = {https://europepmc.org/article/MED/42926865},
note = {CC BY},
}
RIS
TY - JOUR
TI - Intradural cauda equina hemangioblastomas: Surgical management and long-term outcomes at a von Hippel-Lindau referral center.
AU - Roberto Doria-Medina
AU - Nicolas Neidert
AU - Marc Hohenhaus
AU - Christine Julia Gizaw
AU - Kilian Rhein
AU - Athina Ganner
AU - Romina Hohenhaus
AU - Jürgen Beck
AU - Jan-Helge Klingler
PY - 2026
DA - 2026/10/12
JO - Brain & spine
DO - 10.1016/j.bas.2026.107479
UR - https://europepmc.org/article/MED/42926865
AB - Introduction Spinal hemangioblastomas (sHBLs) are rare vascular tumors occurring sporadically or in association with von Hippel-Lindau (VHL) disease. Intradural cauda equina sHBLs are uncommon and understudied.Research question We aimed to describe their surgical treatment and outcomes at a VHL referral center.Methods We retrospectively analyzed a prospectively maintained, surgically ascertained VHL/HBL registry of 237 patients who underwent 565 procedures for 804 resected HBLs between January 1997 and July 2025, with follow-up through February 2026. Functional status was assessed using McCormick grade.Results Intradural cauda equina sHBLs were identified in 32/237 registry patients (13.5%). Eight patients (25%) underwent eight procedures involving ten tumors; the remaining 24 patients had no resection. Reasons for resection were symptoms and/or radiological progression (n = 7), concomitant removal during surgery for another lesion (n = 2), and for diagnostics due to initial presentation (n = 1). All ten tumors were completely resected. At first follow-up, McCormick grade was unchanged in all eight patients, and radicular pain improved in all six symptomatic patients. Median imaging follow-up was 67 months (range, 11-260), with no recurrence was identified. One postoperative cerebrospinal fluid fistula resolved after lumbar drainage. One patient experienced a clinical deterioration after 200 months due to VHL disease burden.Discussion and conclusion Cauda equina sHBLs were infrequently resected in this selected registry. At our center, management is individualized within a structured surveillance program. Surgical decisions are guided by symptoms, radiological progression, lesion location, and clinical context, with favorable outcome. Long-term surveillance at an experienced VHL center remains essential.
SN - 2772-5294
ER -