Intradural extramedullary spinal tumors

Cervical
intradural meningioma presenting as Brown-Séquard syndrome. Axial T1 after gadolinium injection. The mass is homogeneously enhancing and completely intradural, without extension to the foramen.
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Cervical
intradural meningioma presenting as Brown-Séquard syndrome. Axial T1 at the level of C2, showing an intradural extramedullary mass occupying most of the canal and displacing the spinal cord posteriorly and to the right.
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Cervical
intradural meningioma presenting as Brown-Séquard syndrome. Midline sagittal T1, showing an intradural extramedullary anterior mass occupying most of the canal and displacing the spinal cord posteriorly.
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Cervical
intradural meningioma presenting as Brown-Séquard syndrome. Midline sagittal T2WI. Anterior intradural extramedullary lesion. Notice a line of CSF separating the lesion from the cord, displaced posteriorly.
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Cervical
intradural meningioma presenting as Brown-Séquard syndrome. Axial T2WI at the level of C2, showing the intradural extramedullary mass anteriorly, displacing the cord posteriorly and to the right. It has a slight hyperintense signal intensity when compared to the cord parenchyma.
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Cervical
intradural meningioma presenting as Brown-Séquard syndrome. Midsagittal T1WI. Homogeneous enhancement after iv gadolinium administration. Notice the dural tail in the anterior aspect of the dura, adjacent to the mass. There is no cord enhancement.
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Cervical
intradural meningioma presenting as Brown-Séquard syndrome. Coronal T1WI. Homogeneous enhancement after iv gadolinium administration. The mass has well defined margins and does not invade the bone.
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Intradural extramedullary neoplasms are located outside the spinal cord but within the dural sheath.

Epidemiology

The majority (70-80%) of spinal canal tumors are intradural extramedullary .

Clinical presentation

Patients present with signs and symptoms of spinal cord or nerve root compression. Common presentations include weakness, localized back pain, radicular pain, sensory deficits, and gait ataxia.

Pathology

Etiology

The most common primary intradural extramedullary neoplasms are meningioma (20-30%) and schwannoma (15-50%), followed by neurofibroma . Less common entities include solitary fibrous tumor/hemangiopericytoma and malignant peripheral nerve sheath tumor.

Leptomeningeal metastases are frequently seen (5-15%) in the setting of solid tumors (most commonly melanoma, small cell lung cancer, and breast cancer) and hematologic malignancies. In children, the most common intradural extramedullary neoplasms are drop metastases from primary brain tumors (most commonly medulloblastoma). In adults, the most common drop metastases are from glioblastoma.

The category includes primary neoplasms of the cauda equina region, but these have a distinct differential diagnosis, particularly the addition of myxopapillary ependymoma and, less commonly, paraganglioma arising from the filum terminale.

A useful mnemonic can be found here.

Radiographic features

In general, a mass lesion is demonstrated within the spinal canal, sometimes with extension into the neural foramina and extradural paraspinal region (which is more suggestive of a nerve sheath tumor). When there is adequate contrast resolution of the cerebrospinal fluid (CSF) space, the mass can be localized within the dura but outside the spinal cord.

Plain radiograph
  • may be normal
  • may show bone erosion, remodeling, or sclerosis
  • may show a paravertebral mass
CT
  • may show bone erosion, remodeling, or sclerosis
  • may show a paravertebral mass
  • may show tumoral calcifications (especially meningioma)
Myelography
  • deviation of the spinal cord away from mass
  • ipsilateral cerebrospinal fluid (CSF) space enlargement surrounding the mass and contralateral CSF space effacement
  • intradural filling defect outlined by sharp meniscus of contrast (“meniscus sign”)
  • the subarachnoid space is blocked and CSF above the block remains unopacified
MRI

Contrast-enhanced MRI is the modality of choice to fully characterize these masses. The key benefits of MRI are greater sensitivity of detection and the ability to narrow the diagnostic differential by defining signal characteristics and the relationship of the mass to the cord, dura, and nerve roots. In addition, MRI can help in identifying secondary lesions and large feeding/draining vessels.

The appearance will vary depending on the histology and is thus discussed in depth in individual articles (see above list). Distinguishing features include the following :

Differential diagnosis

The differential for intradural extramedullary neoplasms includes non-neoplastic developmental mass lesions in this location:

Uncommon appearances of degenerative disease can mimic a mass:

See also

Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu intradurale extramedulläre Tumoren:
spinale
Arachnoidalzyste
Wikipedia • CC BY-SA 3.0
spinal
paraganglioma
wjso.biomedcentral.com • CC-by-2.0
spinale
Schwannome
www.eurorad.org • CC by-nc-sa-4.0
intramedulläre
spinale Tumoren
ejrnm.springeropen.com • CC-by-4.0
intraspinales
Meningeom
Wikipedia • CC BY-SA 3.0
spinales
Ependymom des Filum terminale
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