title: Primary mixed glioneuronal tumor of the central nervous system in a patient with noonan syndrome: a case report and review of the literature.
abstract: Noonan syndrome is an autosomal dominant condition with variable phenotypic expression. Although an association between Noonan syndrome and various neoplasms has been identified, a relationship with primary glial or neuronal tumors of the central nervous system (CNS) has not yet been established. We describe the case of a 6-year-old male patient with Noonan syndrome and leptomeningeally disseminated low-grade mixed glioneuronal tumor. After a literature review, this case emerges as the third patient to present with Noonan syndrome and primary CNS glial tumor and the first with mixed glioneuronal tumor, indicating the possible association between these individual entities.
authors.affiliation: Children's Center for Cancer and Blood Diseases, Los Angeles, CA, USA.
authors.forename: Courtney B
authors.initials: CB
authors.lastname: Sherman
authors.forename: Afshan
authors.initials: A
authors.lastname: Ali-Nazir
authors.forename: Ignacio
authors.initials: I
authors.lastname: Gonzales-Gomez
authors.forename: Jonathan L
authors.initials: JL
authors.lastname: Finlay
authors.forename: Girish
authors.initials: G
authors.lastname: Dhall
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D001932:Brain Neoplasms / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D002648:Child; D005910:Glioma / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009634:Noonan Syndrome / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D014057:Tomography, X-Ray Computed
chemical_list: D000970:Antineoplastic Agents