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Introduction
  • 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
 

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