Epidemiology A primary central nervous system (CNS) tumor of the spine is classified as intradural extramedullary intradural intramedullary extradural Epidemiology primary CNS tumors are most common intracranially 2-4% occur in the spine 850-1,700 diagnosed in United States annually Intradural Extramedullary Tumors Epidemiology account for 60-70% of all primary CNS spinal cord tumors typically present with: central cord compression radicular pain deep-seated back pain login to view 1 more bullet cauda equina-like symptoms Schwannoma incidence most common intradural tumor, along with meningiomas peak incidence at 40-50 y/o equal occurrence in men and women most are benign login to view 1 more bullet arise from the dorsal nerve roots may be associated with neurofibromatosis type II imaging MRI demonstrates a mass arising from the dorsal nerve root login to view 2 more bullets common locations: login to view 5 more bullets histology difficult to distinguish from neurofibroma biphasic Antoni A (hypercellular) and B (hypocellular) patterns elongated nerve cells in a collagenous background S-100 positive treatment surgical resection postoperative radiation for malignant tumors Meningioma incidence accounts for 25% of all primary spinal cord tumors most often occurs in: login to view 2 more bullets peak incidence during 5th and 6th decades of life risk factors include: login to view 2 more bullets imaging MRI login to view 3 more bullets histology lobulated architecture may contain meningothelial whorls treatment if symptomatic, surgical resection can be curative with complete resection for recurrence, radiation should be considered Intradural Intramedullary Tumors Epidemiology account for 20-30% of all intradural tumors in adults and 50% of intradural tumors in children typically present with: local or radicular pain motor deficits (65%) sensory deficits (60%) sphincter dysfunction (38%) login to view 1 more bullet Ependymoma incidence most common primary intradural and intramedullary CNS tumor classified as: login to view 5 more bullets imaging myxopapillary login to view 9 more bullets cellular login to view 4 more bullets histology cellular with monomorphic cells characteristic rosettes and pseudorosettes treatment gross total resection login to view 1 more bullet Astrocytoma incidence most are benign (30%) peak incidence in the third decade of life most common in children (80-90% of intramedullary tumors) imaging on MRI login to view 4 more bullets histology hypercellular with mitotic figures eosinophilic granules are common treatment gross total resection is difficult due to the infiltrative nature of these tumors radiation is typically used to supplement surgery chemotherapy may be beneficial Extradural Tumors Metastasis extramedullary representative of advanced systemic disease 'drop' metastases from a cranial lesion are possible login to view 1 more bullet intramedullary rare, <1% of all systemic malignancies most commonly from: login to view 2 more bullets imaging rapidly expanding and progressive on MRI intramedullary tumors are commonly found in the cervical region and conus medullaris treatment surgical resection is rare control the metastatic burden with radiation and/or chemotherapy intramedullary metastases are associated with a life expectancy of <3 months Lymphoma incidence rare imaging entire neuroaxis should be evaluated with MRI T2: ill-defined hyperintense lesion with marked homogeneous contrast enhancement less spinal cord enlargement usually found in the cervical spine treatment methotrexate