Neurospine.  2023 Mar;20(1):343-352. 10.14245/ns.2244970.485.

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

• Han B
• Zhang L
• Jia W

• KMID: 2540899
• DOI: http://doi.org/10.14245/ns.2244970.485

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.

Keyword

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