Spinal ependymoma with regional metastasis at presentation

Background: Ependymomas are the most common glial neoplasms in the spinal cord. However, spinal cord ependymomas presenting with regional dissemination along the neuroaxis are rare, with a yet undetermined standard of care. We retrospectively evaluated the management and outcomes of patients who were diagnosed with spinal ependymoma with regional metastases at presentation (SERMP). Methods: Between 2002 and 2012, 16 patients with regionally metastatic spinal ependymomas were diagnosed and treated. The patients were retrospectively divided into two groups according to tumor grading and histological features. Nine patients were diagnosed with myxopapillary ependymomas (MPE), and seven patients were diagnosed with other low-grade ependymomas. Results: With a median follow-up of 46.4 months, 13 out of 16 patients had no postsurgical recurrence/progression of the disease. In three patients, the disease recurred/progressed, leading to death in one patient. There was no correlation between gross total removal (GTR) of the main tumor, or resection of the main lesion and the metastatic foci and increased progression free survival in patients of the MPE group. There was an advantage for patients diagnosed with other low-grade ependymomas. Adjuvant radiotherapy did not prove beneficial. Conclusions: SERMP has a relatively benign course. Achieving GTR of both the main lesion and the metastases is preferable, but should not be achieved at any cost, especially in MPE interfering with the conus medullaris. The benefit of adjuvant radiotherapy remains unproven.