Intraspinal Tumors

• Extradural
• Symptoms: Unrelenting pain at the level of the lesion often worsened by activity, pyramidal tract signs (spasticity, increase DTRs) ascending sensory level, bowel/bladder dysfunction, rapid development of neurologic compromise (days to weeks)
• Adult:
• Metastasis (lung, breast, prostate, thyroid; in 5% no primary source tumor is found) – Usually found in lower thoracic and upper lumbar spine (most red marrow there)
• Reticuloendothelial tumors (lymphoma, multiple myeloma, plasmacytoma)
• Chordoma
• Sarcoma
• Epidural lipomatosis – with steroid use or obesity
• Children:
• Neuroblastoma
• Chordoma
• Sarcoma
• Angiolipoma – lipoma with multiple dilated vascular channels; usually dorsal thoracic spine, F>M, 0-10 years old
• Spinal cord compression – this is a true neurologic emergency!  The longer the deficit is present, the less likely is chance for recovery
• Symptoms: Back pain, leg weakness, bowel/bladder dysfunction usually in patient with known metastatic cancer
• Diagnosis: Emergency MRI Of total spine or myelogram to define lesion
• Metastasis generally spares the disc space, thus differentiating it from infectious spinal processes
• Intradural Extramedullary
• Symptoms: Pain at the level of the lesion, pyramidal tract signs (spasticity, increase DTRs) ascending sensory level, bowel/bladder dysfunction
• Nerve sheath tumor (most common) – can also be extradural (15%), “dumbbell” intradural-extradural (15%), or intramedullary (<1%)
• Location: thoracic = cervical = lumbar
• Usually arise from the dorsal root and present as pain in the radicular distribution of the affected nerve root
• Slow growing with long period from symptoms to diagnosis
• Treatment:  Surgical excision
• Prognosis:  Long-term survival
• Meningioma
• Location:  Thoracic > cervical > lumbar
• Located laterally or ventrilaterally in canal
• Occurs with a 9:1 female to male predominance
• Slow growing with long period from symptoms to diagnosis
• Treatment:  Surgical excision
• Prognosis:  Long-term survival
• Rarely: Paraganglioma, epidermoid, neurenteric cyst (ventral to cord), arachnoid cyst (usually dorsal to cord), hypertrophic neuropathic neuroma (from Dejerinne-Sottas or Charcot-Marie-Tooth)
• Intramedullary
• Symptoms: Band of dissociated sensory loss, poorly localized burning pain (esp at night), segmental loss of DTRs, segmental lower motor neuron dysfunction (weakness, muscle wasting, fasciculations), late involvement of the long tracts
• Ependymoma – 60% of intramedullary tumors
• Most common intramedullary tumor in adults
• T1 iso, T2 hyper
• Two types:
• Cellular – usually in cervical spine, F>M, 40-50 years old, circumscribed but unencapsulated, can be associated with cyst or hemorrhage
• Myxopapillary – in conus or filum, M>F, 20-30 years old, encapsulated, cuboidal cells in papillary arrangement around a mucinous vascular core, 20% destroy bone
• Account for 60% of tumors of cord and filum
• Location:  All segments may be involved, but filum is most common
• Peak incidences in 4^th and 5^th decades
• Myxopapillary variant is most frequent type and accounts for nearly all filum ependynomas
• Treatment:  Surgical excision ± XRT
• Prognosis:  Depends on tumor grade
• Astrocytoma – 30% of intramedullary tumors
• Most common intramedullary tumor in children
• Incidence is approximately 1/25^th of intracranial astrocytomas
• Most occur between the 2^nd and 5^th decades, M=F
• Usually cervical
• Often associated with cyst or syrinx
• Associated with neurofibromatosis
• Two histologic types – fibrillary, diffusely infiltrating and pilocytic
• Treatment:  Surgical excision with or without XRT (depending on grade and extent of resection)
• Prognosis:  Depends on tumor gradeUsually benign (70% overall, 90% children)
• Hemangioblastoma – 5% of intramedullary tumors
• M=F, 20-40 years old
• Consists of capillary vessel-forming endothelial cells and stromal cells
• Sometimes associated with vHL
• Usually associated with syrinx
• Rarely: oligodendroglioma, ganglioglioma, schwannoma, metastasis