J Korean Neurosurg Soc.  2024 Nov;67(6):637-645. 10.3340/jkns.2023.0221.

Prognostic Factors of Spinal Intramedullary Hemangioblastoma : Analysis of Surgical Outcomes and Tumor Characteristics

• Jang HJ
• Moon BJ
• Kim KH
• Park JY
• Chin DK
• Cho YE
• Kim KS

• KMID: 2560405
• DOI: http://doi.org/10.3340/jkns.2023.0221

Abstract

Objective
: Spinal intramedullary hemangioblastoma is a rare and highly vascularized benign tumor. The characteristics of the tumor, its corresponding location, and surgical outcomes remain unknown. The purpose of this study was to identify risk factors and strategies for neurologic deterioration following hemangioblastoma surgery.
Methods
: A comprehensive retrospective analysis was undertaken to evaluate patients who underwent surgical intervention for intramedullary hemangioblastoma at our institution from 1993 to 2022. Patients with at least 1 year of follow-up data were included. The analysis covered patient demographics, pre- and post-operative modified McCormick scale (MMCS), tumor location, and tumor size.
Results
: This study included 25 cases. One-year after surgery, neurological deterioration was observed in five cases (20.0%), and neurological improvement was found in nine cases (36.0%). Five cases were ventrally located, and twelve cases were dorsally located. Ventrally located cases were larger in tumor axial size (p=0.029) than dorsal location tumors, resulting in poorer follow-up MMCS and a higher prevalence of von Hippel-Lindau syndrome (VHL) (p=0.042). Three of them were confirmed to be supplied by the anterior spinal artery. In the case of dorsally located cases, there was no neurologic deterioration.
Conclusion
: In intramedullary spinal cord hemangioblastomas, cases located ventrally had a higher incidence of neurological deterioration following surgery than those located dorsally or in intramedullary extramedullary cases. Ventrally located hemangioblastomas were larger than those in other locations. They were mainly supplied by the anterior spinal artery in VHL patients.

Keyword

Hemangioblastoma; von Hippel-Lindau disease; Spinal cord neoplasms

Figure

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  Fig. 1. A chart illustrating preoperative, immediate postoperative, and 1-year outcomes. Red arrows represent deterioration. Blue arrows indicate improvement in the modified McCormick scale score. Op : operation, FU : follow-up.

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  Fig. 2. A 20-year-old female patient with von Hippel-Lindau syndrome presented with symptoms of pain and weakness in both legs along with sphincter dysfunction. She was diagnosed with a ventral intramedullary hemangioblastoma at T11-12. Her preoperative modified McCormick scale was grade II, which worsened to grade V postoperatively. One year after surgery, it improved to grade III. However, it did not recover to the preoperative level. A : A presyrinx and engorged pial vessels are observed in the T2-weighted image. B and C : A large tumor is observed in T1 contrast-enhanced image, with engorged vessels located anteriorly visible in the axial image. D : Angiography reveals that the feeding artery originated from the anterior spinal artery (arrowhead) with a well-stained tumor (arrow). However, embolization could not be performed.

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  Fig. 3. A 21-year-old female patient without von Hippel-Lindau syndrome presented with symptoms of pain in both legs, sensory changes, and gait disturbance. She was diagnosed with a ventral intramedullary hemangioblastoma at T8. After undergoing segmental artery embolization, she received surgical treatment. Six months post-surgery, her modified McCormick scale grade improved from II to I. A : A large syrinx encompassing the tumor was observed on T2-weighted image. B and C : T1-weighted image with enhanced contrast. A contrast-enhanced intramedullary tumor located ventrally was observed. D : In angiography, staining of the tumor was obvious (arrowhead). A feeding vessel from the radicular artery at T9-10 into the tumor was identified (arrow), leading to its embolization.

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Reference

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