Intramedullary Spinal Cord Tumor
Last Updated: March 27, 2020
Intramedullary spinal cord tumors are rare and challenging entities, comprising 16% to 58% of all primary spinal cord and 2% to 8.5% of all primary central nervous system tumors in the adult and pediatric populations.
Analogous to their extramedullary counterparts, three major histologic subtypes predominate. Together, ependymomas (30-40%), astrocytomas (30-35%) and hemangioblastomas (2-15%) correspond to more than 90% of all intramedullary spinal cord tumors. Other tumors such as gliomas, cavernomas, hamartomas, metastases, and lipomas are rarely encountered.
Several genetic factors have been associated with intramedullary spinal cord tumors. Neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2), and von Hippel-Lindau disease (VHL) are the most common genetic diseases that are prone to cause the development of astrocytomas, ependymomas, and hemangioblasto...