Pediatric Spinal Hemangioblastomas: Clinical Features and Surgical Outcomes of 39 Cases

Abstract

Objective: Spinal hemangioblastomas (HBs) are a rare pathology, especially in the pediatric population. The natural history and long-term outcomes of pediatric patients with spinal HBs remain unclear due to their scarcity.

Methods: A retrospective review of the clinical data and treatment outcomes of children with spinal HBs in our institution from 2012 to 2021 was conducted.

Results: Thirty-nine pediatric patients were included, with an average age of 15.9 ± 2.9 years (range, 8-18 years), and 51.3% were female. Children were more likely to have von Hippel-Lindau (VHL) disease (p < 0.001), a family history of VHL (p < 0.001), multiple symptoms (p = 0.006), a shorter duration of symptoms (p < 0.001), and a larger lesion size (p = 0.004) and volume (p = 0.008) than their adult counterparts. The VHL-associated group of patients was more likely to present with multiple symptoms (p = 0.026), have a family history of VHL (p < 0.001), have multiple HBs (p < 0.001) and have synchronous intracranial lesions (p < 0.001) than the sporadic group. After surgery, 15 patients (38.5%) showed improved clinical outcomes, 17 patients (43.6%) remained unchanged, 4 patients (10.2%) worsened, and 3 patients (7.7%) died of tumor progression. During follow-up, there was a high rate of recurrence and repeated surgery, especially for children in the VHL-associated group.

Conclusion: Pediatric patients with spinal HBs appear to have a higher relapse risk than their adult counterparts. Therefore, life-long follow-up of these patients is necessary, especially for VHL-associated cases. Surgery can benefit children with HBs and should be considered early to avoid irreversible neurological deterioration.

Keywords: Hemangioblastoma; Natural history; Pediatrics; Spinal cord; von Hippel-Lindau disease.

Fig. 1.

Age distribution of 39 children with spinal hemangioblastomas.

Fig. 2.

Magnetic resonance imaging (MRI) features of a 15 years old boy with spinal hemangioblastomas (HBs). Preoperative MRI shows a solid HBs at C3–4 level with iso-hyperintensity on T2-weighted images (A), and significant heterogeneous enhancement on sagittal (B) and coronal (C) view. The HB is associated with complex syrinx and perilesional edema. Computed tomography angiography can reveal the feeding arteries that comes from branches of left vertebral artery and the anterior spinal artery.

Fig. 3.

Kaplan-Meier analyses of recurrence-free survival in pediatric and adult patients with spinal hemangioblastomas.

Fig. 4.

Kaplan-Meier curve for recurrence-free survival (A) and overall survival (B) of sporadic and von Hippel-Lindau (VHL) hemangioblastomas in pediatric patients.