AUTHOR=Carta Roberto , Del Baldo Giada , Miele Evelina , Po Agnese , Besharat Zein Mersini , Nazio Francesca , Colafati Giovanna Stefania , Piccirilli Eleonora , Agolini Emanuele , Rinelli Martina , Lodi Mariachiara , Cacchione Antonella , Carai Andrea , Boccuto Luigi , Ferretti Elisabetta , Locatelli Franco , Mastronuzzi Angela TITLE=Cancer Predisposition Syndromes and Medulloblastoma in the Molecular Era JOURNAL=Frontiers in Oncology VOLUME=10 YEAR=2020 URL=https://www.frontiersin.org/journals/oncology/articles/10.3389/fonc.2020.566822 DOI=10.3389/fonc.2020.566822 ISSN=2234-943X ABSTRACT=

Medulloblastoma is the most common malignant brain tumor in children. In addition to sporadic cases, medulloblastoma may occur in association with cancer predisposition syndromes. This review aims to provide a complete description of inherited cancer syndromes associated with medulloblastoma. We examine their epidemiological, clinical, genetic, and diagnostic features and therapeutic approaches, including their correlation with medulloblastoma. Furthermore, according to the most recent molecular advances, we describe the association between the various molecular subgroups of medulloblastoma and each cancer predisposition syndrome. Knowledge of the aforementioned conditions can guide pediatric oncologists in performing adequate cancer surveillance. This will allow clinicians to promptly diagnose and treat medulloblastoma in syndromic children, forming a team with all specialists necessary for the correct management of the other various manifestations/symptoms related to the inherited cancer syndromes.