From the National Cancer Institute, Pediatric Oncology Branch, Bethesda, MD
Author's disclosure of potential conflicts of interest is found at the end of this article.
Address reprint requests to Katherine E. Warren, MD, National Cancer Institute, Pediatric Oncology Branch, Bldg 10 CRC, Rm 1-3930, Bethesda, MD 20892-1104; e-mail: warrenk{at}mail.nih.gov
Overview: High-grade gliomas represent a significant source of mortality for pediatric patients. These tumors differ from similar-appearing tumors in the adult population in terms of their location, behavior, and molecular pathogenesis. The presentation of children with high-grade gliomas may mimic that of more common childhood illnesses; however, grouping of certain signs and symptoms may make the diagnosis more straightforward. Headache, nausea, vomiting, abnormal gait, poor coordination, and papilledema are the most common presenting signs and symptoms in children. Magnetic resonance imaging (MRI) is the preferred method for evaluation of these tumors. Surgery is performed to relieve symptoms, reduce the tumor burden, and obtain tissue for diagnosis. One exception is the diffuse intrinsic brainstem tumors, which are unresectable and diagnosed based on MRI appearance. Radiation therapy is administered focally after maximum surgical resection, except in the case of a very young child. Doses of 50 to 60 Gy are standard. Although most children are adjuvantly treated with chemotherapy and a few patients may benefit, the role of chemotherapy continues to be a source of debate. Unlike adults, few — if any — randomized studies with a significant number of patients have clearly demonstrated benefit from adjuvant chemotherapy for pediatric patients with glioblastoma; no study has demonstrated a survival improvement for patients with diffuse intrinsic pontine gliomas in the past 3 decades. However, investigators are discovering more about the pathogenesis of these tumors, and novel therapeutic strategies are being investigated, which hopefully, will lead to increased survival, less toxicity, and improved quality of life.
