Management of intracranial ependymoma in children and adults

MRI: magnetic resonance imaging; CSF: cerebrospinal fluid; RT: radiation therapy.

* Intracranial ependymomas are invasive primary brain tumors with the potential to disseminate throughout the central nervous system. Maximal safe resection, adjuvant RT, and chemotherapy all play a role, depending on the age of the patient, location of the tumor, and extent of resection. Because of the complexity involved in these cases, patients with ependymomas should be referred to highly specialized centers whenever possible, particularly for infants with subtotal resections and patients with positive CSF cytology. Refer to UpToDate topic review for further details.

¶ Repeat CSF is suggested for confirmation of a positive cytology, due to the rarity of dissemination at diagnosis and because ependymoma cells have a cytologic appearance that overlaps with nonneoplastic or reactive cells. CSF sampling should be delayed 10 to 14 days after surgery to allow for surgical debris to clear.

Δ Children with completely resected, supratentorial, grade 2 ependymomas have a good prognosis and may be considered for observation rather than immediate RT; this approach is being studied in ongoing clinical trials.

◊ In adults with grade 2 ependymoma, the role of RT after gross total resection is uncertain and treatment decisions should be individualized. Adjuvant focal RT is suggested for adults with grade 3 ependymoma or residual disease after resection of grade 2 ependymoma.

§ Infants who undergo subtotal resection are very high risk, and treatment should be individualized; options after second-look surgery may include further chemotherapy or observation depending on feasibility and results of second-look surgery.

¥ Infants <1 year of age at diagnosis should receive focal RT upon reaching 1 year of age.

‡ Patients with CSF dissemination and/or spinal deposits of an intracranial ependymoma have a poor prognosis, and treatment is individualized. Craniospinal RT is typically indicated after surgery for patients >3 years of age. In younger children, treatment usually consists of chemotherapy with or without focal RT.

† For children >1 year of age, the potential benefits of local RT on local control and long-term survival are generally felt to outweigh the risks. For children age 1 to 3 years, chemotherapy in place of RT should only be in the context of a formal protocol.