Campos Wuilker Knoner, Linhares Marcelo Neves
Division of Neurosurgery, Joana de Gusmão Children's Hospital, Florianopolis, Brazil.
Pediatr Neurosurg. 2010;46(5):385-9. doi: 10.1159/000322897. Epub 2011 Mar 10.
Hemangioblastomas (HB) are rare lesions accounting for 2% of all spinal cord tumors. They are highly vascular, benign tumors that occur either sporadically or in the presence of von Hippel-Lindau disease. Spinal cord HB are usually diagnosed in adult patients and their incidence in early infancy is an extreme rarity.
We present a case of a 1-month-old male with a back deformity and left leg hypomotility. MRI of the spine revealed an intramedullary tumor extending from level T6 to T12.
The tumor was excised completely, using standard microsurgical techniques via a posterior approach. The histological diagnosis was spinal cord HB.
A review of the literature revealed that this neoplasm is composed of 3 major cell types: endothelial cells, pericytes and stromal cells. Complete microsurgical removal is the treatment of choice for spinal cord HB because the tumor is benign. To the best of our knowledge, sporadic spinal cord HB at this age has not been reported so far.
