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.
Postoperative radiotherapy of intra-cranial ependymoma in pediatric and adult patients. Shaw EG, Evans RG, Scheithauer BC, Ilstrup DM, Earle JD. Int J Rad Onc Biol Phys 13(10):1457-62, 1987.
Improved survival in cases of intracranial ependymoma after radiation therapy. Late report and recommendations. Salazar OM, Castro-Vita H, VanHoutte P, Rubin P. J Neurosurgery 59(4):652-9, 1983.
Ependymomas: results of radiation treatment. Garrett PG, Simpson WJ. Int J Rad Onc Biol Phys 9(8):1121-4, 1983.
Intracranial ependymoma: long term results of a policy of surgery and radiotherapy. Vanuytsel LJ, Bessell EM, Ashley SE, Bloom HJ, Brada M. Int J Rad Onc Biol Phys 23(2):313-9, 1992.
Ependymoma: results, prognostic factors and treatment recommendations. McLaughlin MP, Marcus RB Jr., Buatti JM, McCollough WM, Mickle JP, Kedar A, Maria BL. Int J Rad Onc Biol Phys 40(4):845-50, 1998.
The clinical and prognostic relevance of grading in intracranial ependymomas. Ernestus RI, Schroder R, Stutzer H, Klug N. Br. J. of Neurosurgery 11(5):421-8, 1997.
Postoperative radiotherapy of spinal and intracranial ependymomas: analysis of prognostic factors. Stuben G. Stuschke M, Kroll M, Havers W, Sack H. Radiotherapy & Oncology 45(1):3-10, 1997.
Brain Tumor. Part 2 of 2. Black PM. NEJM 324(22):1555-1564, 1991.
The role of radiotherapy in the management of spinal cord glioma. Shirato H, Kamada T, Hida K, Koyanagi I, Iwasaki Y, Miyasaka K, Abe H. Int J Rad Onc Biol Phys 33(2):323-8, 1995.
Spinal ependymomas - the value of postoperative radiotherapy for residual disease control. Sgouros S, Malluci CL, Jackowski A. Br. J. of Neurosurgery 10(6):559-66, 1996.
Practice Guidelines for Brain Tumors - Draft. Krawcczyk, J., Unpublished.