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A 32-year-old man presented with gradually increasing bilateral buttock pain. He had intermittent claudication. Multiple, homogenously enhanced intradural extramedullary lesions at L2-L3 and L5-S1 levels were observed on magnetic resonance imaging. The tumors were debulked and were removed in piecemeal pattern until they had completely been resected. Histopathological examination of the surgical specimens confirmed that both tumors were myxopapillary ependymomas (MPE). MPE presenting as concomitant double tumor at conus-cauda-filum level are very rare. This kind of presentation could not be directly considered as dissemination, since both tumors were in the site of classical origin of MPE. Ten cases of double spinal MPEs have been reported to date. Including the present case, analysis of the 11 patients revealed some facts. There is a male predominance, which is opposite to the ependymomas that are commonly observed in females. Median age at presentation is 15 years. Most pronounced symptom is low back pain that sometimes radiates to lower extremities. Surgical approach was aimed in all tumors, which could be succeeded in all tumors except one. Adjuvant radiation therapy was applied in 5 patients. No recurrences have been reported after surgery or surgery + radiotherapy regimens.
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Spinal ependymoma occupies 40-60% of primary spinal cord tumors and has a feature of intramedullary tumor. The tumor most commonly arises from the central canal of the spinal cord, the conus medullaris or the filum terminale and its pathological features are usually benign. Unlike above characteristics, intra and extramedullary ependymomas are reported very rarely and have wide variety of histological features. We present a rare case of spinal anaplastic ependymoma with an accompanied exophytic lesions extramedullary as well. The tumor was poorly delineated between a spinal cord and the extramedullary components in operative view. After we had confirmed the frozen biopsy as anaplastic ependymoma, the remnant mass embedded in the spinal cord was remained because of its unclear resection margin and the risk of neurological deterioration. She underwent radiotherapy with 50.4 Gy, and there were newly developed mass lesions at the lumbosacral region on the MRI, 14 months postoperatively.
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The intramedullary anaplastic ependymoma rarely occurs in the cervicomedullary junction. A 45-year-old woman had a history of right arm pain for several months. Magnetic resonance imaging (MRI) of the cervical spine demonstrated an intramedullary tumor with syrinx at the cervicomedullary junction. The patient underwent a partial resection at another institute. Neurologic deficit worsened after the first surgery. The follow up MRI showed that the enlarged enhancing tumor and syrinx still existed with the same size and configuration. Complete surgical resection was achieved in the revision surgery. Final histologic examination confirmed the diagnosis of an anaplastic ependymoma, and since complete surgical resection was achieved the patient did not receive adjuvant radiation or chemotherapy. The patient was followed-up periodically at the outpatient department, and at the 7 months follow-up the muscle tone of the right hand was normal but with mild sensory deficit, and the MRI demonstrated no evidence of recurrent disease. Intramedullary anaplastic ependymoma that occur in the cervicomedullary junction which are completely resected may be followed-up without adjuvant radiation or chemotherapy to attain good clinical outcome.
A 36-year-old female patient presented with shoulder pain experienced over a period of one year and progressive weakness in both legs for one month. A magnetic resonance imaging scan revealed an intradural extramedullary (IDEM) fusiform mass about 9.8 cm in length, heterogeneously enhanced at the level of C6-T4 with spinal cord compression. At the time of surgery, the surgeon found an encapsulated IDEM tumor with spinal root attachment. The tumor was completely resected and the histologic diagnosis revealed ependymoma. The patient showed a favorable outcome with no recurrence at the 6-month follow-up. This paper reports a rare case of intradural extramedullary ependymoma with spinal root attachment.
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