Medical Care
Surgery
Surgery is the initial treatment for ependymoma. Patients with total or near total resections have significantly better survival rates than those in whom the resection is grossly incomplete.
Radiotherapy
Radiotherapy remains the standard postoperative therapy. Whether this improves long-term survival remains controversial, especially in infants and those with completely resected tumors, in whom the risk of long-term effects of radiotherapy may outweigh the potential benefits.
Involved-field radiotherapy
In patients with complete resection, 59.4 Gy (infratentorial tumor) and 55.8 Gy (supratentorial tumor) to the original tumor site plus a 1-2 cm margin is recommended.
In patients with subtotal resection, 59.4 Gy to the original tumor site plus a 1-2 cm margin for supratentorial and infratentorial tumors is recommended.
Craniospinal radiation is recommended only for those with leptomeningeal dissemination.
Conformal radiotherapy has been shown to be effective for those with localized, totally resected posterior fossa tumors.
Chemotherapy
The role of chemotherapy is unknown. Clinical trials evaluating the effectiveness of different chemotherapeutic agents for infants and for subtotally resected tumors are ongoing. Promising results have been demonstrated with cyclical oral etoposide in recurrent ependymoma, with response rates as high as 83%. However, induction of secondary leukemia in association with chronic use of this regimen has been reported.
In infants younger than 3 years, studies attempting to delay or omit radiotherapy by using postoperative chemotherapy are ongoing. [12, 13]
Although with current regimens the role of chemotherapy appears limited, measurable responses have been documented. As a single agent, cisplatin has been the most effective in phase II studies (30% response rate). Other platinum compounds such as carboplatin appear less effective.
One study has reported significant responses to vincristine and cyclophosphamide combined.
Trials investigating the effectiveness of preirradiation chemotherapy using platinum or alkylator-based regimens are ongoing in infants and patients with subtotally resected tumors. Preliminary results suggest some benefit in those with anaplastic tumors.
A Children's Oncology Group study evaluated the benefits of preirradiation multiagent chemotherapy in patients with subtotally resected tumors.
Surgical Care
The goal is for gross total or near total resection.
Posterior fossa tumors are approached through a suboccipital craniotomy and may be incompletely resectable because of infiltration of the floor, the fourth ventricle, or brainstem.
Supratentorial tumors tend to be large and may be intraventricular, extraventricular, or both. These tumors have a predilection for the frontal, temporal, and parietal lobes and third ventricle. Approach and degree of resection depend on the tumor's size and location.
A regional, leptomeningeal examination must be examined for metastatic foci.
Surgical estimates of the extent of resection may not be reliable; therefore, postoperative MRI evaluation for residual disease is required and generally should be performed within 72 hours of surgery to avoid confusion with postsurgical inflammation.
Posterior fossa tumors often present with obstructive hydrocephaly and may require the placement of a ventriculoperitoneal shunt if primary resection to reestablish CSF flow is unsuccessful.
The use of "second-look" surgery after 1-2 cycles of chemotherapy before radiotherapy has been studied, and the results are pending.
Ailon conducted a retrospective study of treatment with total resection alone for children with intracranial ependymoma. [14] The study included 26 children who were treated with total resection and who did not undergo subsequent radiation therapy. There were 12 cases of grade II posterior fossa ependymoma (PFE), and 14 of supratentorial ependymoma (STE) in their cohort. Immunostaining was performed to identify the presence of the antigen Ki-67, epidermal growth factor receptor, and the enzyme EZH2. Progression-free survival was inferior for patients with high levels of Ki-67 who were treated with resection alone. No differences in survival were found with regard to PFE and SE for patients who underwent both total resection and radiation therapy. Five-year progression-free survival for patients treated with total resection alone was 60% for those with PFE and 45% for those with STE. Overall survival for patients treated with total resection alone was 70% for those with PFE and 70% for those with STE. Survival was inferior for patients younger than 2 years. The investigators concluded that children who are older than 2 years and who have low levels of Ki-67 and who test negative for EZH2 might be candidates for observation following total resection. [14]
Consultations
Regular team members for the care of all patients include the following:
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Neurosurgeon
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Radiation oncologist
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Pediatric oncologist or neuro-oncologist
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Neurologist
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Neuropsychologist
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Neuroendocrinologist
As a result of the tumor, therapeutic intervention, or both, some patients may require the assistance of occupational and physical therapists for rehabilitation.
Diet and Activity
Diet
No specific dietary restrictions or requirements are indicated.
Patients who develop severe anorexia or weight loss as a result of therapy (particularly infants) may need supplemental nutrition to maintain daily requirements.
Activity
No activity restrictions are required unless dictated by underlying neurological deficits.
Patients with ventriculoperitoneal shunts may be restricted from high-impact sports such as diving.
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MRI showing an ependymoma of the fourth ventricle, compressing the cerebellum and brain stem.
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Sagittal section of an ependymoma of the fourth ventricle.
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Section displaying typical perivascular pseudorosettes of a benign ependymoma.
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Section displaying high cellularity, nuclear atypia, and numerous mitoses characteristic of an anaplastic ependymoma.
