Neurologic Manifestations of Ependymoma Treatment & Management
- Author: Subrata Ghosh, MD, MBBS, MS; Chief Editor: Stephen A Berman, MD, PhD, MBA more...
A multimodality approach that encompasses maximal surgical resection in combination with adjuvant therapy is critical for achieving optimal disease control. Preoperative and perioperative steroids are recommended to help limit edema and alleviate some symptoms.
Surgery remains the most effective therapy for this tumor. It establishes tissue diagnosis, restores normal cerebrospinal fluid flow, and can be used to attempt total removal of the tumor.
A second-look surgery for unexpected residual lesions that are seen on postoperative imaging in an operable location is encouraged in patients with noninvasive, benign histology.
Permanent cerebrospinal fluid (CSF) diversion with ventriculoperitoneal shunt is rarely required.
Postoperative radiation therapy substantially improves survival. Although not proven, some dose-to-response relationship probably exists.
Newer methods that target only the local tumor bed, such as high fractionation radiotherapy or stereotactic radiosurgery, may permit potential dose reduction as compared with conventional radiotherapy. It provides effective tumor control, which may help limit complications such as cognitive dysfunction, growth delay, and hypothyroidism.
Craniospinal axis radiation is recommended only for patients with radiological or pathological evidence of spinal seeding.
Overall, results of chemotherapy are disappointing. Multidrug combinations using VP-16 etoposide, vincristine, CCNU (lomustine), and cisplatin offer limited benefit in patients with recurrent disease.
Gross total resection (GTR) is associated with the lowest rates of mortality, the best overall survival, and the longest progression-free survival rates. Patients with WHO grade II tumors had better overall survival after GTR plus external-beam radiation therapy (EBRT) and better progression-free survival rates than after GTR alone. Patients with WHO grade III tumors had better overall survival after subtotal resection plus EBRT.
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