Neuroimaging of Posterior Fossa Ependymoma in Children

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Fig. 21.1
Origin and classification of ependymomas. (a) Midfloor type. Sagittal T2-weighted image (a) shows tumor almost completely filling the fourth ventricle with a clear-cut cleavage with the vermis (arrowheads). The whole length of the brainstem is visualized. The obex is not visible at the site of origin of the tumor from the floor of the fourth ventricle (arrows). There is tumor extension into the cistern magna and foramen magnum (asterisks). Dilatation of the cerebral aqueduct and superior fourth ventricle (squared area) is due to CSF flow obstruction; notice signal loss due to turbulent flow at this level. (b) Roof type. Sagittal T2-weighted image shows intra-fourth-ventricular mass with lack of demarcation from the inferior portion of the roof (arrowheads) but preservation of a cleavage plane above the fastigium (empty arrows). The entire length of the brainstem is visible, and the obex is preserved (arrow) although the brainstem is globally displaced anteriorly. There is a superiorly located large peripheral cystic component (asterisk). (c, d) Lateral type. Sagittal T2-weighted image (c) shows tumor extending anteriorly (arrows) and displacing the brainstem, which is consequently no longer visible in its entirety along the midline. Tumor also extends inferiorly in the cistern magna and foramen magnum (asterisks). Axial T2-weighted image (d) shows lateral displacement of the brainstem (arrows) and lateral extension into the right Luschka foramen and cerebellopontine cistern (asterisks), leading to encasement of the basilar trunk (b)



(i)

“Midfloor type”: It is a midline tumor arising from the inferior half of the fourth ventricular floor beneath the stria medullaris and representing the most common type; technically, it is a dorsally exophytic brainstem tumor, however usually lacking significant infiltration of the fourth ventricular floor and growing into the fourth ventricle.

 

(ii)

“Roof type”: It is a midline tumor arising from the inferior medullary velum forming the roof of the fourth ventricle.

 

(iii)

“Lateral type”: It is an off-midline tumor that originates into the vestibular area or lateral recess of the fourth ventricle.

 


An accurate localization of the site of origin of posterior fossa ependymomas on neuroimaging carries important prognostic implications. It is ascertained that midline (either midfloor or roof) tumors carry a more favorable prognosis than lateral types, mainly because a gross total resection is more difficult to achieve for lateral masses owing to their deep location and intimate relationships with arteries and lower cranial nerves into the cerebellopontine angle and lateral medullary cisterns [6]. On the other hand, midline lesions are usually less firmly adherent to vital structures, although infiltration of the fourth ventricular floor may limit the extent of surgical removal, thus increasing the chance of recurrence and worsening the prognosis [7].



21.4 Macroscopic Features and Growth Pattern


Ependymomas are nodular, lobulated neoplasms with a macroscopically solid appearance on neuroimaging studies. Usually, nervous tissue infiltration only occurs at the site of origin; the latter may, however, not always be clearly recognizable on imaging and is often ascertained only on surgical observation. Owing to their soft consistency and slow growth rate [8], these masses tend to adapt their shape to that of surrounding structures and grow along existing anatomic structures without infiltrating the adjacent nervous tissues. This results in the typical “plastic development” [9] (Figs. 21.1 and 21.2), a term that describes the tendency of these tumors to spread as ribbonlike extensions throughout the outlet foramina of the fourth ventricle into the subarachnoid spaces of the posterior fossa and cervical spinal canal while maintaining a clear-cut edge toward the adjacent nervous tissue. On ground of their soft consistency, ependymomas also typically encase vessels (both arteries and veins) and nerves. Radiologically, encasement of vessels is defined as a tumor-vessel relationship in which the tumor is inseparable from the vessel wall for 180° or more of the vessel circumference [2] (Figs. 21.1 and 21.3).

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Fig. 21.2
Plastic development of ependymomas. (a) Contrast-enhanced sagittal T1-weighted image shows tumor extending caudally into the foramen magnum and cervical spinal canal (arrows). The tumor has also extended cranially through the prepontine cistern and into the interpeduncular fossa, where it abuts the suprasellar cistern (arrowhead). (b) Contrast-enhanced coronal T1-weighted image shows inhomogeneously enhancing tumor (arrowheads) into the right cerebellopontine angle cistern


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Fig. 21.3
Vessel encasement in ependymomas. (a) Axial T2-weighted image shows lateral ependymoma encasing the basilar trunk (arrowhead) and posterior inferior cerebellar artery (arrow). (b) Sagittal T2-weighted image shows the full length of the basilar trunk (arrowheads) is engulfed by the tumor. (c) Contrast-enhanced coronal T1-weighted image shows encasement of the right vertebral artery (arrow). Notice the caliber of the artery is not reduced. (d) Sagittal T2-weighted image shows cluster of encased small-caliber vessels (arrows). The finding of small encased vessels is almost constant in posterior fossa ependymomas

On presentation, supratentorial hydrocephalus is common due to cerebrospinal fluid flow obstruction from the intraventricular mass. Capped hyperintensities on T2-weighted and FLAIR images around the horns of the lateral ventricles indicate periventricular edema in the setting of progressive uncompensated hydrocephalus. These represent a frequent indication to emergent surgery for shunt placement before the actual surgical operation for tumor excision.


21.4.1 Midline Ependymomas (Midfloor and Roof Types)


These masses are centered on the midline and fill the fourth ventricle to a variable degree. The differentiation of these two types from one another may not be feasible on imaging when the lesion is sufficiently large as to almost completely fill the fourth ventricle. The superior portion of the fourth ventricle is typically free of tumor and forms, together with the overlying dilated cerebral aqueduct, a triangular or oval shape that has been called a “capped fourth ventricle” [8]. By virtue of their midline location, these tumors displace the brainstem anteriorly, but not laterally; thus, the entire length of the brainstem is visible on midsagittal MR images [2]. The obex (i.e., the point on the midline of the dorsal surface of the medulla at the most caudal aspect of the fourth ventricle floor) is seen to be infiltrated in the midfloor type but not in the roof type (Fig. 21.1). Conversely, there is a clear-cut cleavage plane between the posterior surface of the tumor and the vermis in the midfloor type. This morphological appearance on midsagittal images is extremely important in the differentiation from medulloblastomas, which originate from the vermis and project anteriorly in the fourth ventricle without any such separation. In the roof variant, the demarcation between tumor and vermis may not be as conspicuous owing to the tumor’s origin from the ependyma of the inferior medullary velum; however, the cleavage is usually visible at least in the superior portion of the fourth ventricle roof, above the fastigium (Fig. 21.1).

Plastic extension occurs either through the foramen of Magendie or the foramina of Luschka (Figs. 21.1 and 21.2). Extension through the Magendie causes the tumor to grow into the cisterna magna and, often, to herniate into the foramen magnum and cervical spinal canal, surrounding and compressing the medulla and spinal cord [8]. Extension into the lateral recess and Luschka foramen may occur either mono- or bilaterally and may involve the cerebellopontine angle; however, midline lesions typically do not extend further into the prepontine cistern [2].


21.4.2 Lateral Ependymomas


These masses are centered off the midline, as they typically arise in one lateral recess of the fourth ventricle and displace the brainstem laterally, but not anteriorly. This morphological arrangement is the opposite to that of midline (either midfloor or roof) variants. As a consequence of this displacement, the entire length of the brainstem cannot be seen in one single midsagittal plane (Fig. 21.1). Lateral ependymomas often extend through the homolateral Luschka foramen into the cerebellopontine angle and may reach the prepontine cistern, where the mass engulfs the basilar artery (Fig. 21.3), a feature not seen in midline lesions. However, they typically do not extend across the fourth ventricle floor into the contralateral recess and Luschka foramen, unlike the midfloor type. Lateral tumors may also extend inferiorly into the cisterna magna through the Magendie foramen. However, the obex is consistently not infiltrated and can therefore be recognized as a discrete structure [2]. Engulfment of nerves and vessels in the cisternal spaces makes radical excision difficult if not impossible and is the main reason for the worse prognosis as compared with midline tumors.

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Jun 22, 2017 | Posted by in NEUROSURGERY | Comments Off on Neuroimaging of Posterior Fossa Ependymoma in Children

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