Supratentorial malignant gliomas of childhood

Eugene Hwang MD (Dr. Hwang of the Center for Cancer and Blood Disorders, Children's National Medical Center, has no relevant financial relationships to disclose.)
Yoko T Udaka MD ()
Roger J Packer MD, editor. (Dr. Packer of George Washington University; Senior Vice President, Center for Neuroscience and Behavioral Medicine; and Gilbert Endowed Distinguished Professor in Neurofibromatosis and Director, Gilbert Neurofibromatosis Institute and Brain Tumor Institute, Children’s National Health System, has no relevant financial relationships to disclose.)
Originally released April 29, 2003; last updated October 31, 2016; expires October 31, 2019

Overview

Supratentorial malignant gliomas continue to be one of the most difficult types of brain tumors to treat in children. The authors present a comprehensive, yet easily readable, review of the basic principles and practices underlying the causes, diagnosis, treatment, and outcomes of supratentorial malignant gliomas in children. The authors also present the most up-to-date clinical therapeutic, biological, and scientific research advancements in this disease in children.

Key points

 

• Molecular classification of malignant gliomas are examined.

 

• Key differences between adult and pediatric malignant gliomas are discussed.

 

• Tumor grade and gross total resection remain the most important prognostic indicators for a favorable outcome.

 

• Children less than 3 years of age have improved outcomes compared to children greater than 3 years of age.

 

• Survival remains poor, and targeted therapies have had minimal results; however, new clinical trials are currently being investigated.

Historical note and terminology

Brain tumor classification began in the 1920s. The proposed classification system for gliomas was based on the idea that gliomas originated from central nervous system cells arrested at various stages of development (Bailey and Cushing 1926). Recognizing that brain tumors contained heterogeneous cell populations, gliomas were classified on the basis of the morphological appearance and presumed histogenesis of the predominant cell type. Most of the classification schemes have been constructed to various degrees around the conceptual framework introduced by Bailey and Cushing (Russell and Rubinstein 1989; Kleihues et al 1993). Other classifications have proposed that glial cells become progressively more anaplastic, rather than undergo a single neoplastic transformation to malignant glioma (Schoenberg et al 1975). This concept has led to the proposal to simplify brain tumor classification in order to reflect the degree of anaplasia that is present by grading the tumors from grade I (benign) to grade IV (malignant). However, with advancements in tumor biology genetics, it is now known that similar histopathology alone cannot provide prognostic value to the diagnosis. As a result, in 2016, an international collaboration of renowned scientists and clinicians revised the current World Health Organization classifications by including molecular parameters (Louis et al 2016).

Since the 2016 update, the World Health Organization recognizes the following entities as supratentorial malignant gliomas: grade III anaplastic astrocytoma, IDH-mutant, anaplastic oligodendroglioma, IDH-mutant and 1p/19q-codeleted (uncommon amongst pediatric patients), anaplastic pleomorphic xanthoastrocytoma and grade IV glioblastoma, IDH-wild-type (uncommon amongst pediatric patients), glioblastoma, and IDH-mutant. The diagnosis of anaplastic oligoastrocytoma has been reassigned an NOS designation given molecular similarities to either astrocytomas or oligodendrogliomas (Louis et al 2016). An account of the historical evolution of glioma terminology has been previously provided in much greater detail (Zulch 1986).

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