title: Dysembryoplastic neuroepithelial tumors: a prospective clinicopathologic and outcome study of 13 children.
abstract: Dysembryoplastic neuroepithelial tumors (DNETs) are benign intracortical masses that are typically observed in children and young adults and are classified as glioneuronal tumors (WHO grade I). Large and retrospective series of patients with DNETs have been reported, but prospective studies on pediatric cohorts of patients with DNETs have been lacking. In the present study, 13 children (8 boys, 5 girls; age 8-18 years) who had simple (n = 2) or complex (n = 11) partial seizures (seizure duration range, 2-4 years; mean, 1.5 years; mode, 1.2 years) were prospectively enrolled and monitored over 13 years. The DNETs were located in the frontal (n = 2), temporal (n = 9), or occipital (n = 2) cortex. In 11/13 cases, the seizures were resistant to drug therapy, and all the children had surgery consisting of extended lesionectomy coupled with neuronavigation. Pathology examination revealed cortical dysplasia (n = 8), glial nodules (n = 11), calcification (n = 4), cellular atypia (n = 3), endothelial proliferation (n = 1), perivascular inflammation (n = 3), and meningeal involvement (n = 6). All children were seizure free throughout postsurgical follow-up of 2-11 years. This first prospective study with follow-up monitoring of a childhood population with DNETs confirms, on a long-term basis, that the coupled strategy of extended lesionectomy and neuronavigation has good outcome for long-term seizure control.
authors.affiliation: Division of Child Neurology, Department of Pediatrics, University La Sapienza, Rome, Italy.
authors.forename: Alberto
authors.initials: A
authors.lastname: Spalice
authors.forename: Martino
authors.initials: M
authors.lastname: Ruggieri
authors.forename: Salvatore
authors.initials: S
authors.lastname: Grosso
authors.forename: Alberto
authors.initials: A
authors.lastname: Verrotti
authors.forename: Agata
authors.initials: A
authors.lastname: Polizzi
authors.forename: Gaetano
authors.initials: G
authors.lastname: Magro
authors.forename: Rosario
authors.initials: R
authors.lastname: Caltabiano
authors.forename: Piero
authors.initials: P
authors.lastname: Pavone
authors.forename: Francesca
authors.initials: F
authors.lastname: Del Balzo
authors.forename: Nunzio
authors.initials: N
authors.lastname: Platania
authors.forename: Paola
authors.initials: P
authors.lastname: Iannetti
mesh_terms: D000293:Adolescent; D001932:Brain Neoplasms / Q000150:complications / Q000473:pathology* / Q000601:surgery; D002540:Cerebral Cortex / Q000473:pathology* / Q000601:surgery; D002648:Child; D004569:Electroencephalography; D005260:Female; D006801:Humans; D008297:Male; D018302:Neoplasms, Neuroepithelial / Q000150:complications / Q000473:pathology* / Q000601:surgery; D011446:Prospective Studies; D012640:Seizures / Q000209:etiology / Q000473:pathology* / Q000601:surgery; D016896:Treatment Outcome