Brain Tumors and Syndromes in Children

Abstract

(Brain) tumors are usually a disorder of aged individuals. If a brain tumor occurs in a child, there is a possible genetic susceptibility for this. Such genetic susceptibilities often show other signs and symptoms. Therefore, every child with a brain tumor should be carefully evaluated for the presence of a ?tumor predisposition syndrome.? Here, we provide an overview of the various central nervous system tumors that occur in children with syndromes and of the various syndromes that occur in children with brain tumor. Our aim is to facilitate recognition of syndromes in children with a brain tumor and early diagnosis of brain tumors in children with syndromes. Diagnosing tumor predisposition syndromes in children may have important consequences for prognosis, treatment, and screening for subsequent malignancies and nontumor manifestations. We discuss pitfalls in clinical and molecular diagnoses, and the consequences of diagnosing a hereditary disorder for family members. Our improved knowledge of cancer etiology is increasingly translated into management strategies in syndromes in general and will likely lead in the near future to personalized therapeutic approaches for tumor predisposition syndromes.

Pediatric High Grade Gliomas in the Context of Cancer Predisposition Syndromes

“…It is important to distinguish case reports from real increased risk for PHGG. Such CPS where there is no current data of increased risk include BRCA [ 28 , 33 ], tuberous sclerosis [ 87 ], multiple enchondromatosis [ 85 ], Fanconi anemia [ 23 ], Beckwith Widemann syndrome and more [ 12 ]. Adult syndromes can cause HGG but are rare in childhood.…”

Cancer Predisposition Syndromes Associated With Pediatric High-Grade Gliomas

“…There have been some anecdotal reports of pHGG in other syndromes (65), such as tuberous sclerosis (66), Beckwith-Wiedemann and Fanconi Anemia (67). However, these case reports do not prove a real increased risk for pHGG.…”