hrp0082p2-d3-498 | Endocrine Oncology | ESPE2014

A Rare Brain Tumor in Noonan Syndrome: Report of Two Cases

Pellegrin Maria Chiara , Tornese Gianluca , Cattaruzzi Elisabetta , Blank Eva , Kieslich Matthias , Ventura Alessandro

Background: Noonan syndrome (NS) is a congenital polymalformative disorder caused by aberrant up-regulated signalling through RAS GTPase. Although NS is associated with hematologic malignancies, no predisposition for neuronal tumors was reported so far.Objective and hypotheses: We describe two cases of young patients with NS and dysembryoplastic neuroepithelial tumor (DNET). This is the first case series reporting a rare type of intracranial tumor in NS....