Supratentorial primitive neuroectodermal tumor in children: clinical features, treatment outcome and prognostic factors

Childs Nerv Syst. 1999 Aug;15(8):377-83. doi: 10.1007/s003810050418.


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).

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Cerebellar Neoplasms / complications
  • Cerebellar Neoplasms / pathology*
  • Cerebellar Neoplasms / therapy*
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Male
  • Nervous System Diseases / etiology
  • Neuroectodermal Tumors, Primitive, Peripheral / complications
  • Neuroectodermal Tumors, Primitive, Peripheral / pathology*
  • Neuroectodermal Tumors, Primitive, Peripheral / therapy*
  • Prognosis
  • Treatment Outcome