Prognostic Factors in Childhood Intracranial Ependymomas: The Role of Age and Tumor Location

Abstract

Despite several clinical reports on intracranial ependymomas in children, the factors which affect prognosis, and the possibility that certain combinations of factors might limit survival, are still a matter of debate. Between 1976 and 1996 we operated on 35 children with intracranial ependymomas. Postoperative irradiation was given to 27 patients, with associated chemotherapy in 6 cases. Mean follow-up was 62 months. In 12 patients a 5-year follow-up was possible. In October 1996, 18 patients (51.4%) were still alive, the longest disease-free follow-up being 20 years, and the shortest 8 months. We analyzed the prognostic relevance of eight factors. For each factor, different subgroups were distinguished and compared as follows: age at diagnosis (<4 vs. ≥4 years), sex, tumor location (supratentorial vs. infratentorial), tumor size (<4 vs. 4–7 vs. >7 cm), surgical removal (total vs. subtotal), histology (low-grade vs. anaplastic), morphology (solid vs. cystic), adjuvant therapies (treatment vs. no treatment). Two-way contingency tables were made to identify associations between variables. The only significant association was between age and tumor location (p = 0.022): in children under 4, tumors were almost invariably located in the posterior fossa (9 out of 10 cases) with a clear preference for the lateral recess (8 cases). Other correlations were not significant. Kaplan-Meier survival curves were compared to assess the prognostic relevance of each factor. Survival was significantly lower for children under 4, for those with posterior fossa tumors, and for patients with residual tumor (p < 0.05). A multivariate analysis compared variables which significantly affected survival, revealing that age is the most important factor affecting prognosis (p < 0.05), while tumor location and surgical removal do not add any significance to the effect of age on survival. We conclude that age has the strongest prognostic relevance in childhood intracranial ependymomas, while the effect of tumor location on survival may be related to the high incidence of lateral recess ependymomas in younger children.

The prognostic value of histological grading of posterior fossa ependymomas in children: a Children's Oncology Group study and a review of prognostic factors

“…Many studies had Grade II ependymomas that were up to two to three times more frequent than Grade III tumors. 3,4,6,9,11,14,15,17,20,22,24,25,30 In some studies this ratio was almost equal, 13,19,[33][34][35] while in others there were far more Grade III than Grade II ependymomas. 12,26,28,29,31,32 In one study of ependymomas in children under 5 years of age, the authors reported eight Grade II and 60 Grade III ependymomas.…”

“…Among 18 studies where the effect of age on survival was analyzed, 12 studies reported age at diagnosis as an independent prognostic factor. 4,7,[9][10][11]13,20,22,24,25,33,34 Six of these studies considered the cut-off for age groups as 3 years, two as 4 years, and one as 5 years of age. In one study, age was calculated as a continuous variable, and in another study of children younger than 3 years, the cut-off between two age groups was 2 years of age (24 months).…”

“…22 Tumor location was reported to significantly affect prognosis in six studies. [17][18][19]24,26,31 In all the studies with positive findings, posterior fossa tumors had a far less favorable outcome, but only three of these studies provided a multivariate analysis in which location still remained a significant prognostic factor 24,26,31 In the remaining 19 studies, location was either not analyzed as a prognostic factor or was found not to influence survival.…”

Pediatric Ependymoma: Biological Perspectives

“…Historically, children below 3 years of age and infratentorial ependymomas have been associated with a poor outcome (6,(22)(23)(24). It remains unclear whether this reflects tumor biology, the surgical inaccessibility of posterior fossa tumors, which are more prevalent in younger children, or the avoidance of adjuvant radiotherapy in early life resulting from concerns about long term clinical sequelae (4,14,23,25,26).…”

9p21 and 13q14 dosages in ependymomas. A clinicopathologic study of 101 cases

“…The World Health Organization (WHO) classification of these tumors, based on histology, divides them into grade I (myxopapillary), grade II (conventional), and grade III (anaplastic) ependymomas. Histologic grading of these tumors does not predict clinical behavior as accurately as other gliomas, [1][2][3][4][5][6] although there are some studies that show a significant association between tumor grade and prognosis. [7][8][9][10] Thus far, the only consistent prognostic variables include extent of surgical resection, with tumor location and patient's age also having some significance.…”