Pediatric Supratentorial High-Grade Gliomas

Mandeep S. Tamber, M.D., James T. Rutka, M.D., Ph.D., F.R.C.S.(C)

Disclosures

Neurosurg Focus. 2003;14(2) 

In This Article

Abstract and Introduction

The purpose of this review is to highlight some of the pertinent concepts and controversies surrounding the diagnosis and treatment of pediatric supratentorial high-grade gliomas. Unlike the adult counterparts, pediatric high-grade gliomas are likely derived from distinct cytogenetic and molecular alterations. Surgery has been shown to play a role in extending patient survival. Some success is associated with the provision of chemotherapy. Radiotherapy remains an important adjunct in children older than age 3 years. The challenges involved in improving the poor prognosis of children in whom these very aggressive tumors have been diagnosed will be discussed, as well as some of the novel approaches being investigated to improve patient survival and quality of life.

A growing body of evidence suggests that high-grade gliomas in pediatric patients manifest properties that clearly distinguish them from their adult counterparts. Some of the most striking differences between them lie in their differential response to conventional treatment modalities. Whereas adjuvant chemotherapy has been shown to exert only a modest survival benefit when undertaken in adult patients, chemotherapy combined with surgery and radiotherapy is associated with a much more robust survival advantage in children.[12] Several longitudinal cooperative group studies have demonstrated that in addition to the favorable response of childhood high-grade gliomas to chemotherapy, a significant correlation exists between the extent of resection and outcome.[6,12,50] The authors of recent literature reviews focusing on adult patients have failed to demonstrate a similar association.[24,30,42] Finally, it is becoming clearer that the difference between pediatric and adult high-grade gliomas extends well beyond clinical response to treatment and into the realm of molecular cytogenetics and the pathways that ultimately give rise to the adult and pediatric variants of these tumors.[1,4,39,44,47,48] An unfortunate characteristic shared by pediatric and adult high-grade gliomas, however, is their universally poor prognosis: in children with GBM the 5-year survival rates range from 5 to 15%, whereas those with AA fare slightly better, with a 20 to 40% long-term cumulative survival probability.[28,35,46]

The purpose of this brief review is to summarize the epidemiology, clinical presentation, histopathological features, treatment principles, and outcome of pediatric malignant gliomas. The characteristics of childhood and adult high-grade gliomas will be compared and contrasted wherever possible. We will conclude this article with a discussion of some of the recent novel therapeutic strategies.

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