INTRACRANIAL EPENDYMOMAS OF CHILDHOOD - LONG-TERM OUTCOME AND PROGNOSTIC FACTORS

A DETAILED OUTCOME analysis was performed on 40 children with intracra nial ependymomas treated at our institution between 1975 and 1993 to i dentify those factors that were predictive of overall and progression- free survival. Three patients (7.5%) who were treated in the first 5 y ears of the study died within 3 months of surgery and were excluded fr om further outcome assessments. Eight (22%) of the 37 patients who sur vived the perioperative period had evidence of leptomeningeal dissemin ation at presentation, on the basis of either imaging (three children) and/or cytological (six children) results. The 5- and 10-year progres sion-free survival rates among these 37 patients were 45.1 and 36.1%, respectively; overall survival rates were 57.1 and 45.0%, respectively . The site of progression was local in 17 of 19 patients with progress ive disease. Three factors were found to have a significant associatio n (P less than or equal to 0.05) with the outcome on both univariate a nd multivariate analyses: 1) the extent of the resection, 2) the age o f the patient at diagnosis, and 3) the duration of the symptoms before diagnosis. The 5-year progression-free and overall survivals were 8.9 and 22%, respectively, among patients who had evidence of residual di sease on postoperative imaging studies, compared with 68 and 80% rates among patients with no apparent residual disease (P = 0.0001 and P < 0.0001, respectively). Patients younger than 3 years fared significant ly worse than older children (5-year progression-free and overall surv ival rates of 12 and 22%, respectively, in the younger children versus 60 and 75% in older children (P = 0.003 and P = 0.01, respectively). In addition, patients with a duration of symptoms before diagnosis of <1 month had a worse outcome than those with a more protracted course (5-year progression-free and overall survival rates of 33 and 33%, res pectively, versus rates of 53 and 64%, respectively (P = 0.02 for both ). Neither the finding of evidence for dissemination at presentation n or the detection of anaplastic histological features (e.g., dense cell ularity or high numbers of mitoses) were associated with a significant ly worse outcome in this series. The combination of variables that had the strongest association with both favorable and unfavorable outcome s was the combination of the age of the patient and the resection exte nt. Only 2 of 17 patients older than 3 years with gross total resectio ns have died, whereas 13 of 20 children who were either younger than 3 years or had radiologically incomplete resections have died (P < 0.00 01). These results provide a strong rationale for achieving a complete or ''nearly complete'' tumor resection, when such a procedure is safe and feasible. More intensive adjuvant therapy regimens are needed to improve the otherwise dismal prognosis among children younger than 3 y ears and among those children in whom a complete resection is not poss ible.