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Semin Neurol 2018; 38(01): 041-049
DOI: 10.1055/s-0037-1620275
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

What's New in Grade II and Grade III Gliomas?

Julie J. Miller
1   Department of Neurology, Stephen E. and Catherine Pappas Center for Neuro-Oncology, Massachusetts General Hospital Cancer Center, Boston, Massachusetts
,
Wolfgang Wick
2   Neurology Clinic, Heidelberg University Medical Centre and Neuro-Oncology Programme, National Centre for Tumour Diseases, Heidelberg, Heidelberg, Germany
› Author Affiliations
Further Information

Publication History

Publication Date:
16 March 2018 (online)

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Abstract

The majority of World Health Organization grade II and grade III gliomas harbor heterozygous mutations in the metabolic enzyme isocitrate dehydrogenase 1 (IDH1), and tumors with an IDH wild-type status show molecular features of a glioblastoma and simply may constitute a separate disease entity. This discovery has led to a profound shift in the way that gliomas are classified and, consequently, how treatment decisions are made. We will review the current understanding of IDH-mutant gliomagenesis and the preclinical models being used to investigate the underlying biology of these tumors and to explore new therapeutic options for these patients. We further summarize the results of recent pivotal trials addressing treatment of grade II and grade III gliomas and highlight promising IDH-mutant-specific therapies on the horizon.

Keywords

isocitrate dehydrogenase - 2-hydroxyglutarate - 1p/19q co-deletion - CATNON - CODEL
 

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