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Author: Chi-Shing Zee, MD, Chief of Neuroradiology, Professor, Departments of Radiology and Neurosurgery, University of Southern California School of Medicine

Chi-Shing Zee is a member of the following medical societies: American Society of Neuroradiology

Coauthor(s): Moasheng Xu, MD, Professor of Radiology, Zhejiang Province Chinese Medical College; John L Go, MD, Assistant Professor, Department of Radiology, Section of Neuroradiology, Keck School of Medicine, University of Southern California Medical Center; Armen Hovanessian, MD, Clinical Instructor, Department of Radiology, University of Southern California School of Medicine

Editors: Jeffrey L Creasy, MD, Associate Professor, Associate Section Head, Division of Neuroradiology, Director, Neuroradiology Fellowship, Department of Radiology, Vanderbilt University; Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand; Val Runge, MD, Robert and Alma Moreton Centennial Chair in Radiology, Professor, Editor-in-Chief of Investigative Radiology, Department of Radiology, Scott and White Clinic and Hospital; Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute; L Gill Naul, MD, Professor and Head, Department of Radiology, Texas A&M University College of Medicine; Chair, Department of Radiology, Chief, Section of Magnetic Resonance Imaging, Scott and White Memorial Hospital and Clinic

Author and Editor Disclosure

Synonyms and related keywords: intradural extramedullary tumor

Background

Meningiomas are the second most common tumor in the intradural extramedullary location, second only to tumors of the nerve sheath. Meningiomas account for approximately 25% of all spinal tumors. Approximately 80% of spinal meningiomas are located in the thoracic spine, followed by cervical spine (15%), lumbar spine (3%), and the foramen magnum (2%). Most intradural spinal tumors are benign and potentially resectable. The prognosis after surgical resection is excellent.

Pathophysiology

Spinal meningiomas are often located laterally or dorsolaterally in the thoracic spine. Meningiomas of the cervical and foramen magnum tend to be located ventral to the spinal cord. They are believed to arise from the arachnoid cluster cells located at the entry zone of the nerve roots or at the junction of dentate ligaments and dura mater, where the spinal arteries penetrate. For this reason, lateral tumors are more common than dorsal and ventral lesions. Most meningiomas are intradural and extramedullary. Occasionally, they can be purely extradural (7%) or intradural and extradural (6%).

Compression of the cord by the meningioma can cause deterioration of neurologic function. Improvement of neurologic findings can be expected after resection of the tumor.

Spinal meningiomas differ from intracranial meningiomas by their slightly greater proclivity for psammomatous change. In general, histopathologic features of spinal meningiomas are similar to their intracranial counterparts. Meningotheliomatous and transitional features are most common in spinal lesions. Spinal meningiomas are typically globoid, and they vary in consistency depending on the extent of calcification. Multiple meningiomas are rare (2%) and most often associated with neurofibromatosis type II.

Frequency

United States

Intradural spinal tumors can be classified as intramedullary or extramedullary. The incidence of intradural spinal tumors is approximately 3-10 cases per 100,000 population. In children, 50% of intradural lesions are extramedullary, and 50% are intramedullary, whereas in adults, 70% are extramedullary, and 30% are intramedullary.

Mortality/Morbidity

Meningiomas and schwannomas and/or neurofibromas are the most common intradural extramedullary spinal tumors. These benign lesions usually produce an insidious onset of clinical symptoms, which are characterized by myelopathy and radiculopathy, respectively. As tumors grow, the symptom complex may merge, and significant neurologic deficits, including paraplegia, may develop.

Resection of spinal meningiomas can result in excellent recovery, even in patients with notable preoperative deficits. The surgical morbidity rate is low because surgical resection of a meningioma can easily be accomplished by means of simple laminectomy. The recurrence rate is substantially lower than that seen in an intracranial lesion. This observation may be secondary to the greater resectability of spinal meningiomas compared with intracranial lesions. Factors associated with poor outcome include calcified tumors, ventrally located lesions, age (ie, elderly patients), duration and severity of symptoms, subtotal resection, and an extradural component to the tumor.

Sex

Meningiomas most frequently affect women, with a 4:1 female-to-male ratio.

  • Spinal meningiomas are typically seen in women older than 40 years. Most spinal meningiomas in women occur in the thoracic spine.
  • Although meningiomas of the spine occur in males, they do so throughout the spinal canal without a predilection for the thoracic spine.

Age

  • Meningiomas are typically seen in women in the fifth and sixth decades.
  • Approximately 3-6% of spinal meningiomas occur in children. Spinal meningiomas in children usually are associated with neurofibromatosis.

Anatomy

Spinal meningiomas often are located laterally or dorsolaterally in the spinal canal. They are believed to arise from the arachnoid cluster cells, and therefore, they are located at the entry zone of the nerve roots or the junction of the dentate ligaments and dura mater. Most meningiomas are intradural and extramedullary in location. The spinal cord is typically compressed and displaced away from the lesion. The subarachnoid space above and below the mass lesion is widened, with cerebrospinal fluid capping the lesion from above and below. On occasion, they can be purely extradural (7%) or intradural and extradural (6%).

Clinical Details

Symptoms produced by meningiomas are secondary to their broad dural attachment and the gradual growth of the tumor with compression of the cord. The clinical course may be insidious, and symptoms are often confused with symptoms of other lesions of the spine, peripheral nervous system, and thorax. The duration of symptoms may span 6-23 months. Because meningiomas do not arise from nerve root sheaths, as do schwannomas, they typically result in myelopathic rather than radiculopathic findings.

On physical examination, sensory and motor deficits are seen almost equally. A high incidence of Brown-Sequard syndrome is seen, with ipsilateral paralysis, decreased tactile and deep sensation, and a contralateral deficit in pain and temperature sensation. This finding is most likely secondary to the high incidence of laterally positioned meningiomas. With substantial growth of the tumors, clinical findings may merge. Patients most frequently complain of regional back pain, especially at night, followed by sensorimotor changes and, eventually, bowel and bladder dysfunction.

Preferred Examination

MRI with intravenous injection of gadolinium-based contrast agent is the preferred examination. If MRI is not available, CT myelography may demonstrate the lesion.

MRI can provide information regarding tumor signal intensity and contrast enhancement. Because MRI has multiplanar imaging capability, the extra-axial location of a meningioma is illustrated clearly.

Gadolinium-based contrast agents (gadopentetate dimeglumine [Magnevist], gadobenate dimeglumine [MultiHance], gadodiamide [Omniscan], gadoversetamide [OptiMARK], gadoteridol [ProHance]) have recently been linked to the development of nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD). For more information, see the eMedicine topic Nephrogenic Fibrosing Dermopathy. The disease has occurred in patients with moderate to end-stage renal disease after being given a gadolinium-based contrast agent to enhance MRI or MRA scans. As of late December 2006, the FDA had received reports of 90 such cases. Worldwide, over 200 cases have been reported, according to the FDA. NSF/NFD is a debilitating and sometimes fatal disease. Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin; yellow spots on the whites of the eyes; joint stiffness with trouble moving

orstraightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness. For more information, see the FDA Public Health Advisory or Medscape.

Limitations of Techniques

MRI is contraindicated in patients with pacemakers, certain types of aneurysm clips, metallic foreign bodies in the eye and elsewhere. Patients with claustrophobia may have difficulty holding still in the MRI unit long enough to complete the examination.



Astrocytoma, Spine
Dermoid Tumor, CNS
Ependymoma, Spine

Other Problems to be Considered

Spine, schwannoma
Spine, intradural metastasis
Spine, dermoid, teratoma
Spine, exophytic astrocytoma
Spine, exophytic ependymoma



Findings

Plain radiographic findings are usually normal. On occasion, localized widening of the canal may be seen. In rare cases, spinal meningiomas may have an extradural component, which can cause enlargement of the neuroforamen. Calcifications are rarely visible on plain radiographs (1-5%) and usually psammomatous in nature.

Degree of Confidence

On plain images, the degree of confidence is low.



Findings

CT scans obtained without the intravenous injection of contrast material occasionally demonstrate a hyperattenuating lesion resulting from psammomatous calcification or dense tumor tissue. CT scans obtained with the intravenous injection of contrast material may show a homogeneous enhancing tumor.

Myelography or CT myelography is required to demonstrate the intradural extramedullary location of the mass.

The spinal cord is displaced away from the lesion and usually compressed. A sharp meniscus is seen where the contrast agent caps the lesion from above and below. The subarachnoid space on the side of the lesion is widened.

Degree of Confidence

On CT, the degree of confidence is moderate.



Findings

MRI demonstrates the intradural extramedullary location of meningiomas. Lesions are usually isointense to spinal cord on both T1-weighted and T2-weighted images. Lesions are sometimes hypointense on T1-weighted images and hyperintense on T2-weighted images. Homogeneous intense enhancement of the lesion is seen after an intravenous injection of gadolinium-based contrast agent.

Most spinal meningiomas demonstrate broad-based dural attachment. On occasion, a densely calcified meningioma may demonstrate hypointensity on both T1-weighted and T2-weighted images. The spinal cord is displaced away from the lesion and usually compressed. The subarachnoid space above and below the lesion is widened, and a meniscus capping the lesion may be seen.

Degree of Confidence

On MRI, the degree of confidence is high.

False Positives/Negatives

A meningioma with intradural and extradural components occasionally mimic a nerve sheath tumor, or a nerve sheath tumor with a predominant intradural component may mimic a meningioma. However, nerve sheath tumors usually have hyperintensity on T2-weighted images, whereas meningiomas usually are isointense to the spinal cord on T2-weighted images. Most meningiomas are lateral or dorsal, whereas most nerve sheath tumors are ventral. Furthermore, a mass lesion with both intradural and extradural components is most likely to be a nerve sheath tumor.



Findings

Intraoperative ultrasonography may be performed after laminectomy as a tool to localize the mass, which is usually echogenic.



Findings

Spinal angiography usually shows a hypervascular lesion with tumor blush. More important, spinal angiography is performed to identify the location of the artery of Adamkiewicz, which is usually in the lower thoracic region, most frequently on the left side. Care must be taken not to damage the artery during surgery because the artery supplies the spinal cord. Spinal angiography is performed for surgical planning if the meningioma is in the lower thoracic region, regardless of the side of the meningioma.



In highly vascular spinal meningiomas, preoperative embolization with particles may be performed to minimize blood loss during surgery. However, this procedure is usually not necessary because highly vascular spinal meningiomas are uncommon.



Media file 1:  Intradural extramedullary meningioma in the lower thoracic region (same patient as in Images 2-4). Sagittal nonenhanced T1-weighted MRI demonstrates an isointense mass compressing the lower spinal cord.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  MRI

Media file 2:  Sagittal T2-weighted MRI shows an isointense mass lesion that compresses the cord (same patient as in Images 1, 3, and 4).
Click to see larger pictureClick to see detailView Full Size Image
Media type:  MRI

Media file 3:  Sagittal T1-weighted contrast-enhanced MRI demonstrates an intensely enhancing intradural extramedullary mass lesion with dural attachment (same patient as in Images 1, 2, and 4).
Click to see larger pictureClick to see detailView Full Size Image
Media type:  MRI

Media file 4:  Axial T1-weighted contrast-enhanced MRI shows a right-sided intradural extramedullary enhancing mass compressing and displacing the cord to the left (same patient as Images 1-3).
Click to see larger pictureClick to see detailView Full Size Image
Media type:  MRI

Media file 5:  Spinal meningioma with intradural, extramedullary, and extradural components. Sagittal nonenhanced T1-weighted MRIs show a lesion isointense to the spinal cord (same patient as Images 6-9). Low signal intensity is seen involving the C6 vertebral body, consistent with bony sclerosis.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  MRI

Media file 6:  Axial T2-weighted MRI shows a mixed-intensity lesion on the right side of the spinal canal extending through the right neuroforamen (same patient as in Images 5 and 7-9). Curvilinear low signal intensity in the mass lesion results from calcification. The spinal cord is compressed and displaced to the left side.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  MRI

Media file 7:  Sagittal contrast-enhanced T1-weighted MRIs show an intensely enhancing mass compressing the cervical cord (same patient as in Images 5, 6, 8, and 9). Enhancement of the posterior portion of the C5 vertebral body results from tumor invasion.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  MRI

Media file 8:  Coronal T1-weighted MRI shows a dural-based enhancing mass lesion with a small component extending into the right neuroforamen (same patient as in Images 5-7 and 9).
Click to see larger pictureClick to see detailView Full Size Image
Media type:  MRI

Media file 9:  Sagittal reformation nonenhanced CT scan shows a calcified mass in the spinal canal (same patient as in Images 5-8).
Click to see larger pictureClick to see detailView Full Size Image
Media type:  CT



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Meningioma, Spine excerpt

Article Last Updated: Feb 9, 2007