Intramedullary Ependymoma with Associated Holocord Syringomyelia – A Rare Case with Limited Treatment Options

Objective: We present a rare case of an intramedullary ependymoma WHO grade 2 presenting with holocord syringomyelia and discuss special features of adequate diagnosis and treatment options. Case presentation: A 79 years old male patient presented with progressive gait ataxia and back pain. Previous operation of lumbar disc herniation and stenosis of the cervical spine did not improve the symptoms. Delayed MRI of the spinal cord showed extensive growth of an intradural mass with accompanied holocord edema. Biopsy of the intradural tumor at the level T9 revealed an Ependymoma WHO grade 2. Complete tumor resection was not possible; hence radiotherapy remained as the only adjuvant treatment option. Conclusion: Diagnosis and adequate treatment of spinal ependymomas with accompanied holocord edema and /or syringomyelia harbour some difficulties. The presented case shows a very rare clinical entity with distinct extension and associated severe progressive neurological deterioration. Microsurgical treatment techniques reached their limits in this case.