Ependymoma in Adults

​Revised: June 2014

Classically, tumour grade and extent of surgery have been thought to be the most important prognostic factors (1,2,3,4). More recently, other factors, including tumour site (5), radiation therapy (6)and initial performance status (7) have been found to impact on survival.

For both intracranial and spinal cord primary lesions, as complete a resection as possible is attempted (8). Progression-free survival is improved if radiation therapy is given, with doses of at least 45 Gy being employed (7).

The optimal radiation volume for intracranial primary lesions is controversial. Regardless of disease grade or degree of resection, post surgical therapy should consist of local radiotherapy 50-55 Gy. Disseminated disease is uncommon but should receive craniospinal radiation, with a dose of approximately 35-40 Gy to the craniospinal axis, and a boost of approximately 15-20 Gy to the intracranial primary site and 10 Gy to drop metastases in the spine (12).

For spinal ependymomas, the use of adjuvant post-operative radiation has been advocated after less than total resection of low-grade lesions (9). However, this has recently been questioned, with some suggesting an expectant policy with possible repeat surgery when complete resection is not achieved (10). A randomized trial would be useful in this situation.

There is no indication that chemotherapy is useful in the treatment of primary ependymomas in adults. Recurrent disease is often treated with palliative chemotherapy approaches including platinum based regimens, temozolomide and/or etoposide.

References:

  1. Shaw, E.G., Evans, R.G., Scheithauer, B.C., Ilstrup, D.M. & Earle, J.D. (1987). Postoperative radiotherapy of intra-cranial ependymoma in pediatric and adult patients. International Journal of Radiation Oncology - Biology - Physics 13(10):1457-62.

  2. Salazar, O.M., Castro-Vita, H., VanHoutte, P. & Rubin, P. (1983). Improved survival in cases of intracranial ependymoma after radiation therapy. Late report and recommendations. Journal of Neurosurgery 59(4):652-9.

  3. Garrett, P.G. & Simpson, W.J. (1983). Ependymomas: results of radiation treatment. International Journal of Radiation Oncology - Biology - Physics 9(8):1121-4.

  4. Vanuytsel, L.J., Bessell, E.M., Ashley, S.E., Bloom, H.J. & Brada, M. (1992). Intracranial ependymoma: long term results of a policy of surgery and radiotherapy. International Journal of Radiation Oncology - Biology - Physics 23(2):313-9.

  5. McLaughlin, M.P., Marcus, R.B. Jr., Buatti, J.M., McCollough, W.M., Mickle, J.P., Kedar, A. & Maria, B.L. (1998). Ependymoma: results, prognostic factors and treatment recommendations. International Journal of Radiation Oncology - Biology - Physics 40(4):845-50.

  6. Ernestus, R.I., Schroder, R., Stutzer, H. & Klug, N. (1997). The clinical and prognostic relevance of grading in intracranial ependymomas. British Journal of Neurosurgery 11(5):421-8.

  7. Stuben, G., Stuschke, M., Kroll, M., Havers, W. & Sack, H. Postoperative radiotherapy of spinal and intracranial ependymomas: analysis of prognostic factors. Radiotherapy & Oncology 45(1):3-10.

  8. Black, P.M. (1991). Brain tumor. Part 2 of 2. New England Journal of Medicine 324(22):1555-1564.

  9. Shirato, H., Kamada, T., Hida, K., Koyanagi, I., Iwasaki, Y., Miyasaka, K. & Abe, H. (1995). The role of radiotherapy in the management of spinal cord glioma. International Journal of Radiation Oncology - Biology - Physics 33(2):323-8.

  10. Sgouros, S., Malluci, C.L. & Jackowski, A. (1996). Spinal ependymomas - the value of postoperative radiotherapy for residual disease control. British Journal of Neurosurgery 10(6):559-66.

  11. Krawcczyk, J. Practice Guidelines for Brain Tumors - Draft. Unpublished.​