Abstract

Brain tumors in infants account for less than 10% of all pediatric nervous system tumors. They include tumors diagnosed in fetal age, neonatal age and in the first years of life. Among these, high-grade gliomas (HGGs) are a specific entity with a paradoxical clinical course that sets them apart from their pediatric and adult counterparts. Currently, surgery represents the main therapeutic strategy in the management of these tumors. Chemotherapy does not have a well-defined role whilst radiotherapy is rarely performed, considering its late effects. Information about molecular characterization is still limited, but it could represent a new fundamental tool in the therapeutic perspective of these tumors. Chimeric proteins derived from the fusion of several genes with neurotrophic tyrosine receptor kinase mutations have been described in high-grade gliomas in infants as well as in neonatal age and the recent discovery of targeted drugs may change the long-term prognosis of these tumors, along with other target-driven therapies. The aim of this mini review is to highlight the recent advances in the diagnosis and treatment of high-grade gliomas in infants with a particular focus on the molecular landscape of these neoplasms and future clinical applications.

Highlights

  • Brain tumors in infants are rare entities and their definition is still debated

  • We have discussed in the introduction how the definition of infantile tumors, the clinical characteristics of infantile high-grade gliomas (iHGGs), is still a debated issue

  • Infantile brain tumors are a rare entity in pediatric oncology and their characterization is far Infantile brain tumors are a rare entity in pediatric oncology and their characterization is far from being complete

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Summary

Introduction

Brain tumors in infants are rare entities and their definition is still debated. Most authors define them as brain tumors occurring in children in the first years of life, including tumors diagnosed during fetal development, neonatal age and under the age of one year [1]. Other authors consider “brain tumors in infancy” as tumors diagnosed until three to five years of age defining a continuous category of infants and very young patients [2]. Infant brain tumors account for around 10% of all pediatric brain tumors [1] and around half of them (5%) occur in the first six months of life [2]. Diagnostics 2020, 10, 648 review, we have focused on children under one year old as most statistics present in medical literature use this limit for the definition of infantile tumors. Where the statistics refer to a different age group this has been specified in the text

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