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EPENDYMOMA

Ependymomas arise from the cells that line the Ventricles (Cerebrospinal Fluid spaces within the Brain) and the Central Canal of the Spinal Cord. Seventy (70%) percent of Ependymomas that affect the Brain, occur in children. In the Spinal Cord, however, about 96% occur in adults. This type Brain tumors tends to occur in the Posterior Cranial Fossa (the lower back portion of the Brain). About 10% of the Brain tumors will spread to the Spinal Cord through the Cerebrospinal Fluid.

There are a number of varieties of Ependymoma recognized by the World Health Organization.

  1. The Papillary Ependymoma (the most common) occurs in the Brain and Spinal Cord.
  2. The Myxopapillary Ependymoma occurs only at the bottom of the Spinal Cord (a region called the "Filum Terminale".)
  3. The Subependymoma is a common finding at autopsy and a rarely require surgical intervention.
  4. Finally there is the Anaplastic Ependymoma, a more aggressive tumor.

INTRAMEDULLARY EPENDYMOMA

These are tumors that arise from Ependymal Cells within the substance of the Spinal Cord and are the most common Intramedullary Tumor in adults. These tumors, which affect men and women equally, occur throughout life, but are more frequently seen in the middle adult years.

FIGURE 1A (Left): MRI Scan (Gadolinium Enhanced-Sagittal View) of a C2-C4 Intramedullary Ependymoma (Arrow) in a 33 year old Male.

Figure 1B (Right): MRI Scan (Transaxial View at C2-3-Same Patient) There is a thin "rim" of Spinal Cord surrounding the tumor (Curved Arrow.)

TREATMENT

Surgery

The usual treatment for this Spinal Cord Tumor is surgical removal. Modern Minimally Invasive Microsurgical Techniques have been effective in advancing current treatment plans.

Figure 2A (Left): Intra-operative x-ray (Same Patient as Figures 1A&B) for the precise positioning of the Minimally Invasive Medtronic "X-tube" retractor in preparation for a Bilateral C2-C4 Laminectomy -Unilateral Approach.

Figure 2B (Right): Operative Photo (Same patient) of the Minimally Invasive Retractor in position through a 26 millimeter right paramedian incision.

Figure 3A (Left): Post-operative CT Scan (Same Patient) Double-ended Arrow indicates the Right sided Laminectomy at C3. The Broad Arrows correspond to the Horizontal Arrows in Figure 3B (Right). This is a 3-D CT reconstruction of the Cervical Spine in this patient. The Curved Arrows indicate the upper & lower limits of the Laminectomy.

Figure 4 (Above): Operative Photo-Same Patient as Figures 1 & 2) A Minimally Invasive C2-C4 Bilateral Laminectomy (Unilateral approach) allowed sufficient exposure. Here, the Dura Mater (Straight Arrows) has been opened. The surface anatomy of the Spinal Cord is distorted by the pressure exerted from within the Spinal Cord by the large Intramedullary Ependymoma.

Figure 5: Operative Photo (High Magnification-Same Patient.) The entire tumor has been removed permitting an unusual view of the interior of the Spinal Cord. In order to accomplish this tumor resection, a short incision was made through the Posterior surface of the Spinal Cord recognizing that this may well result in a permanent, although limited, sensory deficit. Note the intact Anterior Spinal Artery visible in the extreme depth of the tumor resection cavity.

Compare this view with the Pre-operative MRI Scan (Figures 1A & B) and the Post-operative CT Scan images in Figures 3A & 3B to understand the Minimally Invasive surgical approach.


Figure 6 (Below): MRI Scan (C3 Level-Same Patient as Figures 1-7) at 5 weeks post-operative.

The Spinal Cord (Curved Arrow) appears to have re-expanded after the Tumor was completely removed. (Compare this to Figure 1B.) The Minimally Invasive Laminectomy Unilateral surgical avenue (Double-ended Arrow) is visible here and can be compared to Figures 3A & 3B.

Figure 7 (Right): Photo of patient at his 5-week post-operative office visit.

He is fully ambulatory with normal control of bowel & bladder function. He still has significant weakness in his Right Deltoid muscle (resulting in difficulty raising his arm above his head) and in the Right hand. These weaknesses have continued to improve fairly quickly.

Immediately post-operatively, he was quadriparetic (weak in all 4 extremities) with the Right side worse than the Left. He made rapid progress and was transferred to a Regional Rehabilitation Facility where he continued to improve to full ambulation and capable of self care.

These patients are followed, on a long-term basis, with MRI Scan. Absent evidence of recurrence of tumor, further treatment (Chemotherapy &/or Radiation) is not required.


Radiation Therapy

Radiation treatment (preferably in the form of Stereotactic Radiosurgery) may be required in patients where there is evidence of persistent or recurrent tumor.

Chemotherapy

Chemotherapy may be necessary for some patients.

FILUM TERMINALE EPENDYMOMA

These tumors differ from other Ependymomas by their location within the Filum Terminale, a neurologically non-functioning structure at the lower-most end of the Spinal Cord.

Figure 8A (Left): MRI Scan (Sagittal View)

L3 level Filum Terminale Ependymoma is a solid mass filling the Spinal Canal (Arrow.)

Figure 8B (Right): MRI Scan (Transaxial View) shows the Tumor infiltrating the Nerve Roots (Curved Arrow).

A Minimally Invasive Surgical resection was followed by Radiation & Chemotherapy.


TREATMENT

Surgery

The role of surgery for Filum Terminale Ependymomas depends on the size of the tumor and its relationship to the surrounding roots of the Cauda Equina. We strongly favor Minimally Invasive Microsurgical with Gross Total Resection when possible. Complete surgical resection can be accomplished with small and moderate-sized tumors that remain well circumscribed within the fibrous coverings of the Filum Terminale and are separable from the Nerve Roots of the Cauda Equina. Large tumors can present significant problems for surgical resection. These tumors have been present for many years and present a risk for spread through the Cerebrospinal Fluid (CSF). Filum Terminale Ependymomas that are large and adherent to many Nerve Roots can make total removal difficult to impossible without considerable damage to the Nerve Roots to which they are densely adherent.

Figure 9A (Left): MRI Scan (Sagittal View) 7B (Right): Axial View-same patient as Figures 1A & B)

2 years post-op resection of infiltrating Filum Terminale Ependymoma

Note how the Nerve Roots have remained "clumped" together (Arrows)

While many can be safely and completely resected, those that cannot or should not be totally removed can be treated with post-operative Radiation Therapy and Chemotherapy.

PROGNOSIS

Even in cases where the Tumor can be removed totally, a recurrence rate of at least 20% can be anticipated.

Biologically aggressive tumors, which are more common in younger patients, can recur early after removal. Radiation therapy is an important adjunct for these patients as well as those in whom a significant amount of tumor is left behind during the operation. Neuro-oncologic chemotherapy is an additional option available in some centers.

Figure 10: MRI Scan Series (Sagittal View)

An L1 & L2 Lumbar Level Filum Terminale Ependymoma (Arrows) in a 19 year old male.

This tumor was completely removed using a Minimally Invasive approach. No further treatment was required.

Figure 11: MRI Scan (Transaxial View-Same patient as Figure 3.) The tumor (Curved Arrow) appears to occupy the entire Spinal Canal.

At surgery, the Spinal Nerve Roots (Arrows point to the "dark" round objects) are EXTERNAL to the Tumor and compressed against the inside walls posteriorly of the Dural covering of the Spinal Canal.


Additional Information

We invite the reader to explore our section Spinal Cord Tumor Case Studies for further examples of spinal Cord Ependymoma cases.

From among our patients with Spinal Cord Tumors, we have selected several to present in this special section in order to illustrate the application of Minimally Invasive Techniques as well as some of the characteristics, differences and challenges encountered with these clinical problems.

An example of a complete resection of a Filum Terminale Ependymoma is contrasted with another case in order to demonstrate the issue of dense adherence to Nerve Roots, which limits the reasonable possibility of complete resectability.


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This page last edited on 2/19

All content ©2016 by Neurosurgical Consultants, P.A.
Author, Martin L. Lazar, MD, FACS
All Rights Reserved. See Usage Notices.