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

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 a ge, sex, size of tumor, M stage, extent of surgical resection, histological features, immunohistochemical labelling indices (Ki-67, p53), and apoptoti c index were assessed. The mean age at diagnosis was 6.8 years, and the mal e-to-female ratio was 18:10. The presenting symptoms in 22 cases were incre ased intracranial pressure and focal neurological deficits. Gross total res ection was achieved in 17 cases, near-total (>90%) resection in 3, and subt otal in 7; biopsy was performed in I case. The mean duration of follow-up w as 37 months. For 25 patients who completed planned adjuvant therapy, the 3 -year survival rate was 73%. Univariate analysis showed that the presence o f 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 showe d statistical significance(P=0.03).