title: Supratentorial primitive neuroectodermal tumor in children: clinical features, treatment outcome and prognostic factors.
abstract: To investigate clinical features, treatment outcome and prognostic factors of pediatric supratentorial primitive neuroectodermal tumors(ST-PNETs), 28 ST-PNET cases were retrospectively analyzed. The prognostic importance of age, sex, size of tumor, M stage, extent of surgical resection, histological features, immunohistochemical labelling indices (Ki-67, p53), and apoptotic index were assessed. The mean age at diagnosis was 6.8 years, and the male-to-female ratio was 18:10. The presenting symptoms in 22 cases were increased intracranial pressure and focal neurological deficits. Gross total resection was achieved in 17 cases, near-total (>90%) resection in 3, and subtotal in 7; biopsy was performed in 1 case. The mean duration of follow-up was 37 months. For 25 patients who completed planned adjuvant therapy, the 3-year survival rate was 73%. Univariate analysis showed that the presence of tumor necrosis (P=0.002) and extent of resection (P=0.04) correlated with survival. Patients with a high Ki-67 labelling index (>10%) tended to have shorter survival (P=0.095). In multivariate analysis, tumor necrosis showed statistical significance(P=0.03).
authors.affiliation: Division of Pediatric Neurosurgery, Seoul National University Children's Hospital, 28 Yongon-dong, Chongno-gu, Seoul 110-744, Korea,
authors.forename: H J
authors.initials: HJ
authors.lastname: Yang
authors.forename: D H
authors.initials: DH
authors.lastname: Nam
authors.forename: K C
authors.initials: KC
authors.lastname: Wang
authors.forename: Y M
authors.initials: YM
authors.lastname: Kim
authors.forename: J G
authors.initials: JG
authors.lastname: Chi
authors.forename: B K
authors.initials: BK
authors.lastname: Cho
mesh_terms: D000293:Adolescent; D002528:Cerebellar Neoplasms / Q000150:complications / Q000473:pathology* / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009422:Nervous System Diseases / Q000209:etiology; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000150:complications / Q000473:pathology* / Q000628:therapy*; D011379:Prognosis; D016896:Treatment Outcome