- Department of Neurosurgery, Kaiser Permanente Medical Group, Sacramento, CA, USA
- Department of Neurosurgery, University of California, San Francisco, CA, USA
- Department of Pathology, Kaiser Permanente Medical Group, Sacramento, CA, USA
Correspondence Address:
Kern H. Guppy
Department of Neurosurgery, Kaiser Permanente Medical Group, Sacramento, CA, USA
Department of Neurosurgery, University of California, San Francisco, CA, USA
DOI:10.4103/2152-7806.84246
Copyright: © 2011 Guppy KH. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.How to cite this article: Guppy KH, Hou L, Moes GS, Sahrakar K. Spinal intradural, extramedullary anaplastic ependymoma with an extradural component: Case report and review of the literature. Surg Neurol Int 30-Aug-2011;2:119
How to cite this URL: Guppy KH, Hou L, Moes GS, Sahrakar K. Spinal intradural, extramedullary anaplastic ependymoma with an extradural component: Case report and review of the literature. Surg Neurol Int 30-Aug-2011;2:119. Available from: http://sni.wpengine.com/surgicalint_articles/spinal-intradural-extramedullary-anaplastic-ependymoma-with-an-extradural-component-case-report-and-review-of-the-literature/
Abstract
Background:There have been 18 reported cases of primary spinal intradural, extramedullary ependymomas reported in the literature. One of the 18 cases had an extradural component and was benign. Our case is the second spinal intradural, extramedullary ependymoma with an extradural component and the first with its initial presentation as an anaplastic ependymoma.
Case Description:A 50-year-old male presented with bilateral upper thigh weakness and thoracic numbness. His exam showed the pin-prick level at T5. Magnetic resonance imaging (MRI) of the thoracic spine showed an enhancing lesion at T5–6 with severe compression of the spinal cord with a dumbbell shape extension of the tumor through the right T5–6 neural foramen. The patient had a laminectomy at T4–T6 with the resection of the tumor. Postoperatively, the patient regained full strength in his lower extremities. Intraoperatively, the tumor was found to be intradural, extramedullary with an extradural component. The tumor was found to be an anaplastic ependymoma.
Conclusions:Even though spinal intradural extramedullary ependymomas are very rare, surgeons must be aware that on MRI, they can be mistaken for meningiomas or nerve sheath tumors especially if there is an extradural component. Our case report is the first intradural, extramedullary ependymoma that is anaplastic and has an extradural component. A review of the literature provides little information on the treatment and prognosis for these tumors especially if they are anaplastic. We propose that the treatment, as done in our case, should be complete resection of the tumor with spinal radiotherapy to the tumor level.
Keywords: Anaplastic ependymoma, extradural, extramedullary, intradural, spinal cord
INTRODUCTION
Ependymomas, although representing only 4–6% of all primary central nervous system tumors, are the most common tumors of glial origin found in the spinal cord.[
CASE REPORT
A 50-year-old male who had no significant past medical history was noted to have progressive weakness of his lower extremities over a 2-week period. He had numbness starting in his mid-abdominal area and radiating down both legs to the top of both feet. He had no lower back or thoracic pain and no urinary or bowel incontinence. There was normal strength in the upper and lower extremities with no difficulty walking on heels and toes; however, he had difficulty in standing from a squat position. Sensory examination revealed decreased pin-prick and temperature sensation from just below the umbilicus down into the feet. There were normal deep tendon reflexes for his upper and lower extremities except for hyper-reflexia of the right knee. He also had bilaterally down-going toes with no clonus.
Magnetic resonance imaging (MRI) of the thoracic spine revealed a well-circumscribed isotense T1/bright T2 homogeneously enhancing intradural, extramedullary mass centered at the T5–6 level eccentric to the right [Figure
The patient underwent laminectomy at T4–T6. An extradural component of the tumor was seen on the right T5–6 foramen [
Sections of the tumor microscopically showed a moderately cellular ependymoma characterized by perivascular pseudorosette formation evidenced by round to irregular nuclei with fibrillar processes extending to and surrounding a central blood vessel. Mild nuclear pleomorphism with a mitotic activity was identified. Focal endothelial proliferation and a few foci of necrosis were seen. Several areas of the ependymoma displayed tanycytic features in which the cells were arranged in fascicles of variable density.
Immunohistochemical analysis showed a strong diffuse immunoreactivity for glial fibrillary acidic protein (GFAP), strong membrane immunoreactivity for CD56, and strong nuclear and cytoplasmic immunoreactivity for S100. The perinuclear dot-like immunoreactivity for the epithelial membrane antigen (EMA) was equivocal as the patchy staining noted was not of a greater intensity as that seen on the negative control study. The neoplastic cells were nonreactive for CD34 and neurofilament. The Ki-67 labeling index was variable with foci of the greatest labeling estimated at 5–15%. The final pathologic diagnosis was an anaplastic ependymoma, WHO grade 3.
Following surgery, the patient received external beam radiation between T3 and T8 with a total dose of 5000 cGy delivered using a three-field arrangement with daily doses of 200 cGy. The patient with 6-month follow-up had no recurrent tumor with normal MRI of the brain, cervical, and lumbar spine. He was neurologically intact except for poor proprioception in lower extremities which was unchanged from the time before initiation of surgery.
DISCUSSION
A review of the 18 reported cases of primary intradural, extramedullary ependymomas of the spinal cord [
The location of the tumor often determines the clinical presentation. Mild to severe limb weakness, urinary disturbance and gait abnormalities mainly due to posterior column compression have been common clinical presentations. In almost all cases, these symptoms were reversed after surgery. The thoracic spine has been the most common location with five cases occurring in the cervical spine[
MRI imaging shows well-delineated intradural, extramedullary masses that homogeneously enhanced after gadolinium administration. One exception was the case of Graça et al.[
Intraoperative findings of these tumors have shown well-circumscribed encapsulated tumors with no dural attachment which can be easily removed from the surface of the spinal cord. We have found five exceptions.[
Treatment for the 18 cases varied and depended on the pathology seen on initial presentation or recurrence. Benign tumors were treated with complete surgical resections with no adjunctive treatment. This was similar to the treatment of benign intramedullary cord ependymomas reported by Brotchi et al.[
The hypothesis for the formation of these intradural, extrameduallary ependymomas is that the heterotopic glial tissue is left in the intradural space as the neural tube closes.[
CONCLUSIONS
Primary spinal intradural, extramedullary ependymomas are very rare with only 18 reported cases. This tumor can be confused with meningiomas and nerve sheath tumors due to its similarity in MRI findings. Our case report is the first intradural, extramedullary ependymoma that is anaplastic and has an extradural component. The review of the literature provides little information on the treatment and prognosis for these tumors especially if they are anaplastic. We propose that the treatment, as done in our case, should be complete resection of the tumor with spinal radiotherapy to the tumor level.
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