Imaging in Brain Ependymoma

Updated: Jan 23, 2014
  • Author: William Jeffery Klein, MD; Chief Editor: Eugene C Lin, MD  more...
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Overview

Overview

Ependymoma is a central nervous system (CNS) neoplasm composed of glial cells that have differentiated along ependymal lines. These lesions occur most commonly in the ependymal lining of the ventricles, but they also arise in the filum terminale and the central spinal cord. [1, 2, 3, 4] See the image below.

Case 1a. Ependymoma arising from the fourth ventri Case 1a. Ependymoma arising from the fourth ventricle. A 13-year-old girl with recent onset of headache, nausea, vomiting, and papilledema. Nonenhanced axial computed tomography image demonstrates a large, round tumor arising within the fourth ventricle with attenuating nodular calcifications. Obstructive hydrocephalus is noted with frontal lobe periventricular white matter low attenuation resulting from subependymal cerebrospinal fluid absorption.

Preferred Examination

Radiologic imaging plays a role in both the diagnostic workup and treatment of patients with ependymoma; imaging is essential to assess tumor response to therapy and recurrence. Patients with CNS symptoms routinely undergo cross-sectional imaging. Computed tomography (CT) scanning is often the modality used initially to evaluate for clinical concern for intracranial hemorrhage, mass, or mass effect. A general limitation of CT is radiation exposure. Additionally, the use of iodinated contrast material may sometimes be associated with nausea, vomiting, and rarely anaphylactoid reactions. Limitations of CT with respect to ependymoma include suboptimal anatomic detail.

If a tumor is suspected, magnetic resonance imaging (MRI) is the next study performed. In fact, MRI is the primary imaging modality used in the study of ependymomas. MRI best characterizes CNS tumors, and scan findings should result in an accurate differential diagnosis and frequently a specific diagnosis. CT scanning is a useful preliminary adjunct, particularly to show tumor calcifications. Before the development of cross-sectional and multiplanar imaging, angiography and pneumoencephalography were used to localize brain masses and characterize tumor vascularity.

General limitations of MRI include cost and the need for patient cooperation. Patient motion is a potential cause of considerable artifact. Patients with claustrophobia and some children require preliminary sedation. Another general limitation of MRI is artefact from metallic foreign bodies and medically implanted objects, such as pacemakers. Finally, MRI has limited benefit in the evaluation of cortical bone and the detection of calcium.

Ultrasonography, nuclear medicine studies, angiography, myelography/cisternography, and radiography are of limited use in the workup of ependymoma.

The final diagnosis of ependymoma, as with most CNS neoplasms, is achieved with tissue biopsy; however, when correlated with demographic and clinical features, MRI and CT scan findings can be strongly suggestive of ependymoma. [5]

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Radiography

Radiographic findings are included for historical interest. A study by Barone and Elvidge demonstrated that in 45 pathology proven cases of ependymoma, intracranial calcifications were present in 6 patients. [6] The pineal gland was calcified in 4 patients and was displaced from the midline in 2 patients. Separation of the cranial sutures from raised intracranial pressure occurred in 12 patients. In the 43 patients in whom ventriculography was performed, 41 demonstrated hydrocephalus with identification of the site of obstruction. [6]

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Magnetic Resonance Imaging

MRI is the diagnostic modality of choice in the workup and follow-up observation of intracranial neoplasms, including ependymoma. The most appropriate role for MRI in the treatment of ependymoma is in the detection of tumor and for anatomic guidance for surgical resection and/or radiation therapy. MRI is used to monitor ongoing treatment and to search for recurrence. Although the MRI findings can be of great help in narrowing the differential diagnosis of brain tumors, final diagnosis is achieved through biopsy with histopathologic analysis.

Solid portions of ependymoma are typically isointense to hypointense relative to white matter on short recovery time/echo time (TR/TE) T1-weighted images. The tumor is hyperintense to white matter on long TR/TE T2-weighted images. As many as 50% of ependymomas demonstrate signal heterogeneity, which may indicate calcification, necrosis, methemoglobin, hemosiderin, or tumor vascularity. [7, 8, 9] For example, hyperintense foci on both T1- and T2-weighted images suggest methemoglobin in subacute hemorrhage of 1-4 weeks in duration, whereas hypointense foci on both T1- and T2-weighted images suggest hemosiderin, calcium, or necrosis.

See the images below.

Case 2a. Fourth-ventricle ependymoma in a 63-year- Case 2a. Fourth-ventricle ependymoma in a 63-year-old man with headaches. T1-weighted sagittal image demonstrates an oval, fourth ventricular tumor with hypointense signal (likely from tumoral calcifications, confirmed on gradient echo sequence). Moderate obstructive hydrocephalus of the lateral and third ventricles is noted.
Case 2b. Fourth-ventricle ependymoma. T1-weighted Case 2b. Fourth-ventricle ependymoma. T1-weighted coronal postgadolinium intravenous image. Homogeneous enhancement of a fourth ventricular mass is noted, with extension downward through the foramen of Magendie. Pathologic analysis demonstrated subependymoma.

Punctate calcific foci are difficult to diagnose prospectively by MRI scan but are present in up to 45% of ependymomas. [9, 10]

See the images below.

Case 5a. Anaplastic right frontal lobe parenchymal Case 5a. Anaplastic right frontal lobe parenchymal ependymoma in a 5-year-old girl with seizures. Contrast enhanced T1-weighted axial image demonstrates a heterogeneous mass in the right frontal lobe. There is bright contrast enhancement within the neoplasm and areas of low signal intensity are consistent with calcification.
Case 5b. Anaplastic parenchymal ependymoma. T2-wei Case 5b. Anaplastic parenchymal ependymoma. T2-weighted axial image shows heterogeneous high signal intensity in the right frontal lobe tumor and adjacent vasogenic edema, with low signal intensity calcifications. There was no tumor connection to the lateral ventricle noted on imaging or at the time of surgery. Pathologic analysis demonstrated malignant (anaplastic) ependymoma.

Cystic changes result in high signal intensity on T2-weighted MRIs variably depending on cyst contents.

See the images below.

Case 3a. Anaplastic ependymoma of the lateral vent Case 3a. Anaplastic ependymoma of the lateral ventricle in an 8-week-old girl with lateral ventricular hydrocephalus. Gadolinium-enhanced coronal T1-weighted image demonstrates a large anaplastic necrotic ependymoma of the left lateral ventricular roof with severe mass effect and subfalcine herniation.
Case 3b. Anaplastic ependymoma of the lateral vent Case 3b. Anaplastic ependymoma of the lateral ventricle. Gadolinium-enhanced axial T1-weighted image demonstrates the large anaplastic ependymoma of the left lateral ventricle with severe mass effect, and left-to-right subfalcine herniation.

Signal heterogeneity is a feature useful in distinguishing ependymoma from the more homogeneous medulloblastoma. Calcification and hemorrhagic foci are more typical of ependymoma than medulloblastoma. Additionally, ependymomas are more apt to extend through the foramina of Luschka and Magendie, hence the term “plastic ependymoma.” Similarly, choroid plexus papilloma is more homogeneous than ependymoma and lacks the typical irregular margins of ependymoma.

See the images below.

Case 4a. Ependymoma arising from the fourth ventri Case 4a. Ependymoma arising from the fourth ventricle in a 50-year-old woman with a history of dizziness and nausea, progressive over several years. The lobulated mass in this proton density–weighted sagittal image arises from the fourth ventricle and extends distally through the foramen of Magendie. There is minimal lateral ventricular hydrocephalus. Pathologic analysis demonstrated cellular ependymoma.
Case 4b. The lobulated fourth ventricular mass in Case 4b. The lobulated fourth ventricular mass in this proton density–weighted coronal image extends distally through the foramen of Magendie. Pathologic analysis demonstrated cellular ependymoma.

Enhancement with IV gadolinium is useful in differentiating tumor from adjacent vasogenic edema and normal brain parenchyma. Without intravenous contrast enhancement, T2-weighted images are more reliable in differentiating tumor margins than are T1-weighted images. [10]

Previous cerebral angiography reports describe ependymomas causing displacement of the vein of the lateral recess of the fourth ventricle on cerebral arteriography. [10] This vein normally courses from the transverse and lateral supratonsillar veins along the anterior and lateral aspect of the superior pole of the cerebellar tonsil. It then courses lateral to the cerebellopontine angle, over the brachium pontis, to join the petrosal vein. Ependymoma expanding the fourth ventricle and its lateral recesses can displace this vein posteriorly and laterally.

Supratentorial ependymomas are more commonly located in the brain parenchyma than infratentorial ependymomas, which are often intraventricular. Swartz and colleagues reported that 83% of supratentorial ependymomas were located in the cerebral parenchyma. [11] Supratentorial ependymomas tend to be larger than infratentorial ependymomas, with 94% being larger than 4 cm in one study. [12] In addition, supratentorial ependymomas are often extraventricular and more often have a cystic component, with or without a mural nodule. In these cases, the differential diagnosis includes ganglioglioma, pilocytic astrocytoma and pleomorphic xanthoastrocytoma. In the posterior fossa, medulloblastoma and cerebellar astrocytoma can mimic the appearance of an ependymoma. [6]

Gadolinium-based contrast agents (gadopentetate dimeglumine [Magnevist], gadobenate dimeglumine [MultiHance], gadodiamide [Omniscan], gadoversetamide [OptiMARK], gadoteridol [ProHance]) have 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.

NSF/NFD 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. 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 or straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness. For more information, see FDA Information on Gadolinium-Based Contrast Agents or Medscape.

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Ultrasonography

The role of ultrasonography in the evaluation of ependymoma is limited. Fetal ultrasonography and pediatric transcranial sonography are used primarily as screening tools for some pathologic conditions but can detect hydrocephalus reliably. A study by Han and colleagues demonstrated that 6 of 1528 infants undergoing transcranial ultrasonography had a pathologically proven brain neoplasm. [10] One patient had ependymoma. Ultrasonography demonstrated a solid echoic fourth ventricular mass with localized, well-defined, anechoic cystic areas. [10] These findings are not sensitive or specific for ependymoma.

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