Treatment for spinal myxopapillary ependymoma mainly involves surgical excision of the tumour.
Radiotherapy
If the capsule ruptures or the tumour is not confined to the filum terminale, the mass could infiltrate and adhere to the cauda equina and/or conus medullaris or disseminate via the cerebral spinal fluid 1) 2).
Therefore, adjuvant radiotherapy is recommended when en bloc excision (removal of the entire tumour as one piece) cannot be accomplished 3) 4).
However, the efficacy of radiation therapy has not been established and can result in adverse effects such as radiation myelopathy 5) 6) and residual dysuria 7).
In the series of Tsai et al., the median age at diagnosis was 35 years (range, 8-63 years). Twenty patients (39%) had surgery alone, 30 (59%) had surgery plus radiotherapy (RT), and 1 (2%) had RT only. At a median follow-up of 11 years (range, 0.2-37 years), 10-year overall survival (OS), progression free survival (PFS), and local control (LC) for the entire group were 93%, 63%, and 67%, respectively. Nineteen patients (37%) had disease recurrence, and the recurrence was mostly local (79%). Twenty-eight of 50 patients who had surgery (56%) had gross total resection; 10-year LC was 56% after surgery vs 92% after surgery and RT (log-rank P = .14); the median time of LC was 10.5 years for patients receiving gross total resection plus RT, and 4.75 years for gross total resection only (P = .03). Among 16 patients with subtotal resection and follow-up data, 10-year LC was 0% after surgery vs 65% for surgery plus RT (log-rank P = .008). On multivariate analyses adjusting for resection type, age older that 35 years at diagnosis and receipt of adjuvant radiation were associated with improved PFS (hazard ratio [HR]: 0.14, P = .003 and HR: 0.45, P = .009) and LC (HR: 0.22, P = .02 and HR: 0.45, P = .009).
Postoperative radiotherapy after resection of MPE was associated with improved PFS and LC 8).
Therefore, adjuvant radiotherapy is recommended when en bloc excision (removal of the entire tumour as one piece) cannot be accomplished 3) 4).
However, the efficacy of radiation therapy has not been established and can result in adverse effects such as radiation myelopathy 5) 6) and residual dysuria 7).
In the series of Tsai et al., the median age at diagnosis was 35 years (range, 8-63 years). Twenty patients (39%) had surgery alone, 30 (59%) had surgery plus radiotherapy (RT), and 1 (2%) had RT only. At a median follow-up of 11 years (range, 0.2-37 years), 10-year overall survival (OS), progression free survival (PFS), and local control (LC) for the entire group were 93%, 63%, and 67%, respectively. Nineteen patients (37%) had disease recurrence, and the recurrence was mostly local (79%). Twenty-eight of 50 patients who had surgery (56%) had gross total resection; 10-year LC was 56% after surgery vs 92% after surgery and RT (log-rank P = .14); the median time of LC was 10.5 years for patients receiving gross total resection plus RT, and 4.75 years for gross total resection only (P = .03). Among 16 patients with subtotal resection and follow-up data, 10-year LC was 0% after surgery vs 65% for surgery plus RT (log-rank P = .008). On multivariate analyses adjusting for resection type, age older that 35 years at diagnosis and receipt of adjuvant radiation were associated with improved PFS (hazard ratio [HR]: 0.14, P = .003 and HR: 0.45, P = .009) and LC (HR: 0.22, P = .02 and HR: 0.45, P = .009).
Postoperative radiotherapy after resection of MPE was associated with improved PFS and LC 8).
1) , 5) Sakai Y, Matsuyama Y, Katayama Y, et al. Spinal myxopapillary ependymoma: Neurological deterioration in patients treated with surgery. Spine. 2009;34:1619–1624.
2) , 3) , 7) Nakamura M, Ishii K, Watanabe K, et al. Long-term surgical outcomes for myxopapillary ependymomas of the cauda equine. Spine. 2009;34:E756–760.
4) , 6) Volpp PB, Han K, Kagan AR, Tome M. Outcomes in treatment for intradural spinal cord ependymomas. Int J Radiation Oncology Biol Phys. 2007;69:1199–1204.
8) Tsai CJ, Wang Y, Allen PK, Mahajan A, McCutcheon IE, Rao G, Rhines LD, Tatsui CE, Armstrong TS, Maor MH, Chang EL, Brown PD, Li J. Outcomes after surgery and radiotherapy for spinal myxopapillary ependymoma: update of the MD anderson cancer center experience. Neurosurgery. 2014 Sep;75(3):205-14. doi: 10.1227/NEU.0000000000000408. PubMed PMID: 24818785.