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      Spinal intradural, extramedullary anaplastic ependymoma with an extradural component: Case report and review of the literature

      case-report

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          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.

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          Most cited references25

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          Spinal cord ependymoma: radical surgical resection and outcome.

          Several authors have noted increased neurological deficits and worsening dysesthesia in the postoperative period in patients with spinal cord ependymoma. We describe the neurological progression and pain evolution of these patients over the 1-year period after surgery. In addition, our favored method of en bloc tumor resection is illustrated, and the rate of complications, recurrence, and survival in this group of patients is addressed. We operated on 26 patients (12 male and 14 female) with low-grade spinal cord ependymomas between 1975 and 2001. The median age at diagnosis was 42 years. Tumors extended into the cervical cord in 13 patients, the thoracic cord in 7 patients, and the conus medullaris in 6 patients. Eleven patients had previous surgery and/or radiation therapy. We achieved a gross total resection in 88% of patients, whereas 8% had a subtotal resection and 4% had a biopsy. Only 1 patient developed a recurrence over a mean follow-up period of 31 months. We conclude that radical surgical resection of spinal cord ependymomas can be safely achieved in the majority of patients. A trend toward neurological improvement from a postoperative deficit can be expected between 1 and 3 months after surgery and continues up to 1 year. Postoperative dysesthesias begin to improve within 1 month of surgery and are significantly better by 1 year after surgery. The best predictor of outcome is the preoperative neurological status.
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            Spinal cord tumors.

            Spinal cord tumors are rare; however, every radiologist should be able to recognize and readily identify those lesions often found in younger patients or children [1, 2, 3, 4, 5, 6, 7, 8,9]. Early diagnosis plays an important role in the management of the lesions and interferes with the prognosis and final outcome of the patient [10]. Plain X-ray of the spine and CT are of limited diagnostic value in cases of intramedullary pathology. Magnetic resonance imaging should be performed as soon as possible and as the first technique whenever an intrinsic spinal cord lesion is clinically suspected. Systematic analysis of the MR images together with a basic knowledge of the most common spinal cord tumors encountered, including astrocytomas, ependymomas, and hemangioblastomas, should provide a correct diagnosis and lead to appropriate treatment subsequently.
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              Primary intradural extramedullary ependymoma: case report and review of the literature.

              The authors report the ninth case in the literature of a primary intradural extramedullary ependymoma of the spinal cord. To discuss surgical treatment and the physiopathologic hypothesis of this localization on the basis of the results of the present study and a review of the literature. Ependymoma is a glial tumor known to arise in the central nervous system. Intradural extramedullary location of this neoplasm has been exceptionally described previously. A 43-year-old woman was admitted to the authors' institution with an history of progressive paraplegia. Neurologic examination showed sensory loss below T1 and bladder disturbances. Magnetic resonance imaging revealed an enhanced thoracic intradural extramedullary tumor, extending from T1-T8. No other lesion in the central nervous system was found. Emergency surgical resection was performed. Surgery gave confirmation of an encapsulated extramedullary tumor without attachment to the spinal cord or to the dura mater. Total removal was achieved under microscope. The postoperative course was uneventful, with complete neurologic recovery 3 months later. The patient has been well for 24 months of follow-up evaluation, without evidence of recurrence on magnetic resonance images. Histologic examination revealed the tumor as a benign ependymoma. The encapsulated feature, the lack of attachment to the central nervous system, and the absence of other neoplastic processes within the brain or the spinal cord suggested that this lesion is a primary tumor developed from ectopic ependymal cells.
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                Author and article information

                Journal
                Surg Neurol Int
                SNI
                Surgical Neurology International
                Medknow Publications Pvt Ltd (India )
                2229-5097
                2152-7806
                2011
                30 August 2011
                : 2
                : 119
                Affiliations
                [1 ]Department of Neurosurgery, Kaiser Permanente Medical Group, Sacramento, CA, USA
                [2 ]Department of Neurosurgery, University of California, San Francisco, CA, USA
                [3 ]Department of Pathology, Kaiser Permanente Medical Group, Sacramento, CA, USA
                Author notes
                [* ]Corresponding author
                Article
                SNI-2-119
                10.4103/2152-7806.84246
                3172008
                21918734
                2bcf6f4c-ba8a-42b8-b1a3-98fb81cf295a
                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.

                History
                : 08 July 2011
                : 12 July 2011
                Categories
                Case Report

                Surgery
                spinal cord,extramedullary,anaplastic ependymoma,intradural,extradural
                Surgery
                spinal cord, extramedullary, anaplastic ependymoma, intradural, extradural

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