Intramedullary spinal tumours

Intramedullary spinal tumours are rare, representing 4-10% of all CNS tumours and less than 10% of all paediatric CNS neoplasms 5. They account for 20% of all intraspinal tumours in adults and 35% of all intraspinal tumours in children 8.

A long duration of symptoms prior to diagnosis is typical.

Classification

They can be classified according to many ways:

Associations

Intramedullary spinal neoplasms are more common in patients with neurofibromatosis:

  • ependymomas occur more often in patients with NF2
  • astrocytomas occur more often in patients with NF1

Approximately 70% of intramedullary tumours are associated with cysts 3. Two types of cysts are recognised:

  • tumoural (or intratumoural) cysts
    • contained within the tumour itself
    • typically demonstrate peripheral enhancement
    • may result from necrosis, fluid secretion, or degeneration of the neoplasm
    • need to be resected along with the solid portion of the tumour because there is a high likelihood of neoplastic cells within the cyst wall
    • occurs in association with the following proportion of tumours 5,6,9
      • spinal ganglioglioma: in 46%
      • spinal ependymoma: in 22%
      • spinal astrocytoma: in 21%
      • spinal haemangioblastoma: in 2-4%
  • non-tumoural (or reactive) cysts
    • occur rostral or caudal to the solid portion of the tumour
    • occur due to dilatation of the central canal
    • do not enhance
    • present in 60% of all intramedullary spinal tumours
    • may resolve once the neoplasm is resected

Syringomyelia occurs in approximately 50% of all intramedullary tumours but is most frequently associated with hemangioblastomas 3.

The presentation of intramedullary tumours depends on their size and location. The most common presenting symptoms include back/neck pain, radicular pain, weakness, paraesthesia, gait disturbance and bowel and bladder dysfunction. Brown-Sequard syndrome may occur. An uncommon presentation is acute headache due to subarachnoid haemorrhage 8.

In children, progressive scoliosis may be seen. Motor regression and frequent falls may be the presenting features in young children 7.

Symptoms are usually slowly progressive. Due to their non-specific nature, the diagnosis is often delayed. An exception is intramedullary metastastases, which are diagnosed within one month of symptom onset in up to 75% of cases 6.

Plain film

Widening of the interpedicular distance may be seen in less than 10% of cases

CT

Generally not useful because bony changes are relatively rare.

Myelography
  • the spinal cord can be enlarged
  • gradual subarachnoid space effacement
MRI

3 general characteristics of intramedullary neoplasms are recognised on MRI:

  1. they cause either focal or diffuse spinal cord expansion
  2. they produce high signal intensity on proton density and T2 weighted images
  3. the vast majority show at least some contrast enhancement, in contrast to intracranial neoplasms, even low-grade intramedullary tumours enhance to some degree; however, the absence of enhancement does not exclude an intramedullary neoplasm in the presence of cord expansion

Various lesions may mimic intramedullary tumours. The differential diagnosis of intramedullary tumours includes:

  • vascular lesions
    • cavernous malformation (cavernoma)
      • rounded regions of heterogeneous signal intensity on T1 and T2 weighted images due to blood
      • products of varying ages (“popcorn appearance”)
      • low signal intensity rim on T2 weighted images (haemosiderin)
      • hypointense “blooming” on gradient echo sequences (haemosiderin)
      • may demonstrate minimal enhancement on post-contrast images
      • minimal cord expansion or oedema unless there has been recent haemorrhage
    • dural arteriovenous fistula (DAVF)-(type I AVM) 
      • spinal cord may be normal size or enlarged
      • hyperintense on T2 weighted images (due to oedema)
      • prominent vessels (flow voids) are usually present on the posterior aspect of the cord
      • variable enhancement on post-contrast images
      • typically located in the dorsal aspect of the lower thoracic cord and conus medullaris
    • spinal cord infarction 
      • spinal cord is usually enlarged
      • hyperintense on T2 weighted images and DWI
      • post-contrast enhancement may or may not be present (enhancement is usually present in the subacute stage)
      • signal intensity abnormality may be limited to the central grey matter or may involve most of the cross sectional area of the cord
      • signal abnormality typically extends over multiple vertebral body segments
      • can occur at any location in the cord but has a propensity for the upper thoracic or thoracolumbar regions
      • vertebral body T2 hyperintensity may occasionally be seen (due to concomitant infarction)
  • inflammatory lesions
    • demyelination (e.g. multiple sclerosis)
      • usually no spinal cord enlargement (enlargement is seen in 6-14% cases) 4
      • plaques are generally shorter than two vertebral body segments in length and involve less than half the cross-sectional area of the cord
      • characteristically peripherally located in the dorsal and lateral columns
      • isointense to hypointense on T1 weighted images (unlike brain plaques, cord plaques may not be visible as areas of hypointensity on T1 weighted imaegs)
      • hyperintense on T2 weighted images
      • signal abnormality is typically poorly marginated
      • plaque enhancement correlates with acute lesion activity (usually lasts less than two months)
      • in most patients additional lesions of variable enhancement are present in the brain and spinal cord (MS is solely confined to the spinal cord in 5-24% of patients 4)
    • transverse myelitis
      • variable enlargement of the spinal cord
      • hyperintense on T2 weighted images and iso- or hypointense on T1 weighted images
      • variable enhancement patterns (none, diffuse, patchy, peripheral)
      • lesions commonly extend for 3-4 spinal segments
      • lesions typically occupy greater than two thirds of the cross-sectional area of the cord
      • acute clinical course
    • spinal cord abscess 
      • core is hypointense on T1 weighted images and hyperintense on T2 weighted images
      • rim enhancement
      • cord expansion
      • may show restricted diffusion on DWI
      • may have adjacent vertebral or disc abnormalities
  • spinal cord contusion (acute)
    • isointense to hypointense on T1 weighted images, hyperintense on T2 weighted images
    • cord swelling
    • usually associated with other spinal injuries (osseous, disc, vascular)
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Article Information

rID: 19260
Synonyms or Alternate Spellings:
  • Intramedullary spinal tumour
  • Intramedullary spinal tumors
  • Intramedullary spinal tumor
  • Intramedullary spinal cord tumour
  • Intramedullary spinal cord tumor
  • Intramedullary spinal cord tumors
  • Intramedullary spinal cord tumours

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    Case 1: spinal ependymoma
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    Case 2: spinal ganglioglioma
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