Multiple Spinal Cord Recurrences of an Intracranial Ependymoma after 14 Years
33-year-old male visited with the complaint of sudden dysuria, difficulty in defecation, and progressive paraparesis. He also had a thoraco-lumbar back pain for two months. Bilateral weakness in the leg muscles (grade 4) and hypesthesia/numbness below T12 dermatome were found in the neurological examinations. Micturition was not possible. There were no specific abnormalities in the cranial nerves and upper extremities.\nFourteen years prior, the patient was diagnosed with a fourth ventricle low grade ependymoma. A gross total resection of the tumor with a ventriculoperitoneal shunt procedure was performed. Postoperative radiation therapy was also performed delivering 45 Gy to the posterior fossa and 10 Gy boost to the tumor bed. At initial surgery, the evaluation for spinal metastasis was not considered and prophylactic spinal irradiation was not performed because the intracranial ependymoma was low grade. Follow up was continued for three years, without any sign of tumor recurrences. All the previous medical records were meticulously reviewed.\nThe thoracolumbar MR image taken at a different hospital demonstrated an intradural-extramedullary mass involving T12 and L1 (). The tumor was 5.7 cm in size, dorsal in location, and was compressing the spinal cord. Emergency operation with a routine laminectomy of T12-L1 was performed. A gray-red intradural extramedullary mass was noted and was easily separated from the spinal arachnoid and completely removed without complication. Weakness and voiding difficulties experienced by the patient improved immediately after surgery.\nThe histologic findings of the lesion were consistent with an ependymal tumor with a high Ki-67 mitotic index (up to 60%), moderate nuclear pleomorphism, and tumor necrosis. Based upon the histology, a diagnosis of World Health Organization (WHO) grade III anaplastic ependymoma was made (). A postoperative whole spine MR study revealed multiple small enhancing lesions from C6 to L5 (). A brain MR showed no evidence of residual tumor or tumor recurrence (). No other ventriculoperitoneal shunt pathway-related metastases were found.\nThe patient received a radiation therapy to the whole spine of 36 Gy in 1.5 Gy fractions and with an additional boost of 9 Gy in 1.8 Gy fractions to the thoraco-lumbar tumor site using a 6 MV photon. During radiation therapy, RTOG/EORTC grade 2 hematologic toxicity was observed and resolved without specific treatment. Chemotherapy was reserved for disease progression. The patient's neurologic and clinical status improved and remained stable. Follow up images showed disappearance or a decrease in size of the spinal residual mass after radiation therapy and there was no evidence of tumor progression for three years ().