Post-Doctoral Neurosurgery Research Fellow Johns Hopkins University Johns Hopkins University School of Medicine Baltimore, MD, US
Disclosure(s):
Abdel-Hameed Al-Mistarehi, MD: No financial relationships to disclose
Introduction: Hemangioblastomas are benign, and highly vascular tumors comprise 6% of spinal cord tumors and can significantly impact quality of life. Though more common in adults, HGBs can also present in pediatric populations. Historically, resection has been the treatment of choice. However, recent findings have shown encouraging results with radiotherapy. Herein, we aim to compare survival in adult and pediatric patients treated with surgical resection with or without radiotherapy for hemangioblastoma.
Methods: The National Cancer Database was queried for hemangioblastoma cases between 2004 and 2017. Patient, tumor, and treatment characteristics were collected; the primary outcome was five-year overall survival. The cohort was categorized into adult and pediatric groups.
Results: A total of 768 patients were included in this study, comprising 716 adults and 52 pediatrics. There were no significant differences in sex, race, Charlson-Deyo Comorbidity Classification (CDCC) score, tumor size, treatment modality, and extent of resection between adults and pediatrics. Adults lived closer to the hospital, with a mean distance of 53.2±111.54 miles, than the pediatrics (93.67±213.00 miles) (p=0.02). Length of hospital stay, 30-day readmission, and 30-day or 90-day mortality rates showed no significant differences between the two groups. However, the five-year mortality rate was higher in adults than in children (13.1% vs 3.8%, p=0.05). Kaplan-Meier analysis demonstrated that the mean survival was significantly higher in the pediatric cohort than in adults (p=0.038). Gross total resection (GTR) with or without radiation had the highest survival probability compared to radiation-only (p < 0.001). Meanwhile, the mean survival was significantly lower in patients with either CDCC score of ≥2 or 1 compared to those with a CDCC score of 0 (p < 0.001). Cox regression revealed that CDCC score of ≥2 (HR 3.16; 95%CI 1.73-5.78; p< 0.001) and CDCC score of 1 (HR 1.89, 95%CI 1.10-3.25, p=0.021) were risk factors for mortality, while GTR with/without radiation significantly improved survival (HR 0.30, 95%CI:0.13-0.67, p=0.003 and HR 0.28, 95%CI:0.09-0.85, p=0.024, respectively).
Conclusion : Adults with hemangioblastoma have a higher five-year mortality rate compared to pediatric patients. Additionally, higher CDCC indicates reduced five-year survival whereas gross total resection is linked to substantial improvement in survival.