Pediatric Spinal Vascular Abnormalities: Overview, Diagnosis, and Management

Hemangioblastomas are true benign vascular neoplasms arising from pluripotent mesenchymal stem cells that give rise to vascular endothelial cells and are most commonly found in the cerebellum, spinal cord, brainstem, and retina. These tumors may be isolated sporadic lesions or may be associated with hereditary genetic factors in the case of von Hippel-Lindau (VHL) syndrome. Spinal cord haemangioblastomas constitute 1.1% to 2.4% of all central nervous system tumors105, with the majority being single tumors that present in the fourth decade of life 106. In the pediatric population, sporadic spinal cord hemangioblastomas are exceedingly rare. The prevalence of spinal cord hemangioblastomas in children is increased among those with VHL syndrome. The thoracic cord is the most common site for spinal cord hemangioblastomas, followed by the cervical cord. Although these tumors are benign, they cause disabling symptoms due to spinal cord compression, syringomyelia, or hemorrhage from the tumor itself or from aneurysms that form on tumor-feeding arteries or intra-tumoral vessels.