Neurosurgical Consultants: Nervous System Tumors

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Martin L. Lazar, MD, FACS, FAANS
James E. Bland, MD, FAANS

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EPENDYMOMA

Ependymomas are tumors that arise from Ependymal cells, which make up the thin "lining" of the Ventricular walls of the Brain cavities (called "Ventricles", where Cerebrospinal Fluid [CSF] is manufactured) and the Central Canal of the Spinal Cord. Ependymal cells are the third type of cell in the "Glial" cell series, the others being Astrocytes and Oligodendrocytes.

Ependymomas are six times more common in children compared to adults. The highest age incidence is 1 to 5 years with a second, smaller peak in the mid-30s.

In a manner similar to other "Gliomas", Ependymal Cells can form a tumor known as an "Ependymoma". These tumors can occur within the Brain or Spinal Cord. By virtue of the fact that Ependymomas exist WITHIN THE CEREBROSPIANL FLUID PATHWAYS, they have the potential to spread ("seed") through the CSF (Cerebrospinal Fluid) pathway to other areas of the Nervous System.

The commonest sites of origin are:

Intracranial: Ependymomas account for only 5-6% of all intracranial Gliomas with 69% occurring in children (9% of pediatric Brain tumors). These are usually well circumscribed and "benign" - appearing. They commonly arise in the Floor of the Fourth Ventricle (60-70% are infratentorial in region of the 4th Ventricle).

Figure 2: MRI Scan (Transaxial View-same patient as Figures 1, 4 & 5) 4^th Ventricular Ependymoma. The tumor (centrally located lighter "grey" mass) fills the 4th Ventricle in these images (Arrows).

Spinal: These account for 60% of Spinal Cord Gliomas, with 96% occurring in adults.

Figure 3A (Left): MRI (Transaxial View). The Spinal Canal is filled with Tumor (Arrows.)

Figure 3B (Right): MRI (Sagittal View-same patient.)
The Ependymoma (Arrow) fills the Spinal Canal compressing the Nerve Roots.

Please consult our website section on Spinal Cord Tumors for a thorough review and explanation of the MINIMALLY INVASIVE PROCEURES AVAILABLE FOR THE REMOVAL OF THESE TUMORS.

SYMPTOMS

The symptoms of these intracranial tumors are similar to those of other intracranial masses. This includes headache (80%), nausea/vomiting (75%), ataxia (difficulty with balance) or vertigo (dizziness), and seizures.

DIAGNOSIS

These tumors are primarily diagnosed by CT or MRI scans (See Figures 1 & 2). The diagnosis is then confirmed by microscopic examination of the tumor.

TREATMENT

Surgery

THE GOAL OF SURGERY IS THE RESECTION (removal) of the GREATEST AMOUNT OF TUMOR POSSIBLE, WITHOUT CAUSING ADDITIONAL NEUROLOGICAL DEFICITS.

When invasion of the surrounding Brain is extensive, total excision is not possible, although we have had encouraging long-term experience with the removal of the small portion of tumor that invades the floor of the Fourth (4^th) Ventricle in selected patients. The long term results, in these patients, have been excellent.

Figure 4: Operative Photo
4^th Ventricle Ependymoma
(Same patient as Figures 1 & 2)
The Dura Mater is open showing the 2 Cerebellar Hemispheres. (Upper Arrows)
TUMOR fills the space between the Hemispheres (Middle Arrow)
The lower portion of the Medulla joins the upper portion of the Cervical Spinal Cord (Lowest Arrow)

Figure 5: Post-operative MRI (Gadolinium Enhanced-Sagittal View)
The 4^th Ventricular ependymoma has been completely excised and the 4^th Ventricle and CSF pathways are now open. (Arrow)
Compare this MRI to the Pre-operative study in Figure 1.

Our Experience and Long-term Follow-up
The Neurosurgeons of Neurosurgical Consultants have long term follow-up experience ranging to 25 years after the successful complete Microsurgical Resection of 4^th Ventricle Ependymomas without adjunct Radiation Therapy or Chemotherapy. As an example, one such resection was accomplished in a 20 year old female (who is now 47 years old) and subsequently had three (3) successful pregnancies. Despite the fact that she remains without symptoms and lives in another part of the USA, we continue to follow her with yearly MRI Scans sent to us from her home town. We continue to encourage patients with a history of Ependymomas to adhere to follow-up protocols.

Radiation Therapy

Radiation Therapy may be an important adjunct particularly in cases where incomplete excision is known to have occurred, where there is clear evidence of residual or recurrent tumor or where there are "Drop Metastasis" within the CSF pathways.

Chemotherapy

Chemotherapy is the third arm of a comprehensive effort to prolong and maintain a high quality of life. Chemotherapy has a particularly important role to play in patients where incomplete excision is known to have occurred, where there is clear evidence of residual or recurrent tumor or where there are "Drop Metastasis" within the CSF pathways.

Current Chemotherapy protocols for these tumors have been upgraded to include some of the relatively non-toxic yet aggressive agents. One of our Neuro-oncologists has utilized newer medications such as Temodar (temozolamide), Avastin (bevacizumab, an anti-angiogenesis agent) and CPT-11, either alone (or more commonly) in combination or with other drugs, to produce encouraging results. Additional information regarding these treatments is available at the "Virtual Trials" website. Ultimately we continue to hope for the development of specific gene therapies.

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This page last edited on 2/19