Epidemiology Primary central nervous system (CNS) tumor of the spine is classified as intradural extramedullary intradural intramedullary extradural Epidemiology primary CNS tumors 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 symptoms related to central cord compression radicular pain deep seated back pain most intense at night associated cauda equina-like symptoms Schwannoma incidence most common intradural tumor along with meningiomas peak incidence in 40s-50s equal occurrence between men and women most are benign, with malignant subtypes arise from dorsal nerve root may be associated with neurofibromatosis type II imaging MRI exhibits mass arising from dorsal root T1 shows isotense mass T2 shows hyperintensity common locations: cervical spine (31%) cauda equina (24%) thoracic spine (22%) upper cervical spine (16%) conus medullaris (4%) histology difficult to distinguish from neurofibroma biphasic, Antoni A (hypercellular) and B (hypocellular) pattern elongated nerve cells in collagen background S-100 positive treatment surgical resection post-operative radiation for malignant tumors Meningioma incidence accounts for 25% of all primary spinal cord tumors most often occurs in: women (80%) thoracic spine peak incidence during 5th and 6th decades of life risk factors include: neurofibromatosis type II previous history of radiation imaging MRI well-circumscribed, dura-based lesion on T1, iso- or hypointense on T2, slightly hypertensive with homogenous enhancement with contrast 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% in children typically present with symptoms related to: local or radicular pain motor deficits (65%) sensory deficits (60%) sphincter dysfunction (38%) often initial symptom Ependymoma incidence most common intradural intramedullary primary CNS tumor classified as: benign myxopapillary (40-50%) cellular (42%) malignant anaplastic imaging myxopapillary on MRI, encapsulated lesion in the filum terminale on T1, hypo- or isointense on T2, hyper intense heterogeneous on contrast enhancement other features typically seen are: vertebral body scalloping neural foraminal enlargement scoliosis tumor seeding in the sacrum cellular on MRI, similar to myxopapillary distinguishing characteristic polar cysts hemorrhage histology cellular, monomorphic cells characteristic rosettes and pseudorosettes treatment gross total resection good prognosis, especially if planes are maintained Astrocytoma incidence most are benign (30%) peak incidence in third decade of life most common in children (80-90% of intramedullary tumors) imaging on MRI fusiform appearance with irregular margins On T1, hypo- or isointense On T2, hyper intensive with variable contrast enhancement typically found in cervicothoracic junction in children histology hypercellular, mitotic figures eosinophilic granules are common treatment gross total resection difficult due to infiltrative nature radiation typically used to supplement surgery chemotherapy may be beneficial Extradural Tumors Metastasis extramedullary representative of advancing systemic disease 'drop' metastases from cranial lesion possible spread via CSF intramedullary rare, <1% of all systemic malignancies most commonly from: lung breast imaging rapidly expanding, progressive on MRI intramedullary commonly found in cervical region and conus medullaris treatment surgical resection rare control burden with radiation and/or chemotherapy intramedullary metastases associated with less than 3 month life expectancy Lymphoma incidence rare imaging entire neuroaxis should be analyzed via MRI on T2, ill-defined hyperintense lesion with marked homogeneous contrast enhancement less cord enlargement usually found in cervical spine treatment methotrexate