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Multidisciplinary management of pediatric intracranial ependymoma
1Department of Radiation Oncology & Pediatrics, Case Western Reserve University School of Medicine, University Hospitals & Seidman Cancer Center, Rainbow Babies & Children's Hospital, 11100 Euclid Avenue, Cleveland, OH 44106-6068, USA. david.mansur@uhhospitals.org.
Insights
Pediatric intracranial ependymoma, a rare pediatric brain tumor, is primarily treated with surgery and radiation. The optimal role of chemotherapy remains under investigation in ongoing clinical trials.
Area of Science:
- Pediatric neuro-oncology
- Childhood brain tumors
Background:
- Pediatric intracranial ependymoma accounts for approximately 7% of pediatric brain tumors.
- The rarity of these tumors complicates the establishment of a definitive standard therapy.
Purpose of the Study:
- To review current treatment strategies for pediatric intracranial ependymoma.
- To highlight ongoing research and clinical trial designs addressing treatment controversies.
Main Methods:
- Review of existing data on surgical resection, radiation therapy, and chemotherapy.
- Analysis of current clinical trial designs, including the Children's Oncology Group trial.
Main Results:
- Gross total resection is associated with improved outcomes.
- Postoperative radiation therapy is standard for grade II and III tumors.
- The role of chemotherapy is not yet clearly defined.
Conclusions:
- Surgical resection and radiation therapy form the cornerstone of treatment.
- Further research is needed to optimize chemotherapy's role in pediatric ependymoma treatment.
- Current clinical trials aim to resolve treatment controversies.
Abstract:
Pediatric intracranial ependymoma is a rare disease representing approximately 7% of brain tumors in children aged 15 years or younger. Due to the relative rarity of these tumors, a clear standard therapy has been difficult to establish. The mainstay of treatment is surgical resection and the majority of data demonstrate improved outcome with gross total resection. The standard of care also includes postoperative radiation therapy for most patients with grade II and III tumors. Chemotherapy has been used in many capacities in this disease; however, its optimal role is yet to be defined. Current controversies such as treatment with surgery alone in completely resected tumors, use of chemotherapy for subtotally resected tumors and use of adjuvant postradiation chemotherapy are incorporated into the design of the current Children's Oncology Group clinical trial.
