TY - JOUR
T1 - A multi-institutional retrospective study of intracranial ependymoma in children
T2 - Identification of risk factors
AU - Horn, Biljana
AU - Heideman, Richard
AU - Geyer, Russel
AU - Pollack, Ian
AU - Packer, Roger
AU - Goldwein, Joel
AU - Tomita, Tandori
AU - Schomberg, Paula
AU - Ater, Joann
AU - Luchtman-Jones, Lori
AU - Rivlin, Kenneth
AU - Lamborn, Kathleen
AU - Prados, Michael
AU - Bollen, Andrew
AU - Berger, Mitchel
AU - Dahl, Gary
AU - McNeil, Elizabeth
AU - Patterson, Kathleen
AU - Shaw, Dennis
AU - Kubalik, Michael
AU - Russo, Carolyn
PY - 1999/5
Y1 - 1999/5
N2 - Purpose: The goal of this multi-institutional retrospective study of children with intracranial ependymoma was to identify risk factors associated with unfavorable overall survival (OS) and event-free survival (EFS). Patients and Methods: Clinical data, including demographics, tumor location, spread, histology, details of surgery, radiation treatment, and chemotherapy were collected. Clinical characteristics and univariate and multivariate analyses of risk factors for OS and EFS are presented. Results: Eleven U.S. institutions contributed 83 patients treated from 1987 to 1991. The OS at 5 and 7 years was 57% and 46%, and EFS at 5 and 7 years was 42% and 33%. Patients 3 years of age or younger differed from the older group by more common infratentorial location, less common gross total resection (GTR), and postoperative use of chemotherapy rather than radiation. This younger group of patients had worse survival (P < 0.01) than the older age group. Other than young age, less than GTR and World Health Organization (WHO) II grade 3 histology were significant adverse risk factors for EFS in univariate and multivariate analyses. OS shared the same adverse risk factors except for histology in multivariate analysis, which was only of borderline significance (P = 0.05). Progression at the original tumor location, present in 89% of patients, was the major pattern of tumor recurrence. Adjuvant chemotherapy in the group older than 3 years or craniospinal radiation in M0 patients did not significantly change EFS. Conclusions: Adverse outcome in childhood intracranial ependymoma is related to age (3 years or younger), histology (grade 3), and degree of surgical resection (less than GTR). New approaches, particularly for local tumor control in younger patients, are needed to improve survival.
AB - Purpose: The goal of this multi-institutional retrospective study of children with intracranial ependymoma was to identify risk factors associated with unfavorable overall survival (OS) and event-free survival (EFS). Patients and Methods: Clinical data, including demographics, tumor location, spread, histology, details of surgery, radiation treatment, and chemotherapy were collected. Clinical characteristics and univariate and multivariate analyses of risk factors for OS and EFS are presented. Results: Eleven U.S. institutions contributed 83 patients treated from 1987 to 1991. The OS at 5 and 7 years was 57% and 46%, and EFS at 5 and 7 years was 42% and 33%. Patients 3 years of age or younger differed from the older group by more common infratentorial location, less common gross total resection (GTR), and postoperative use of chemotherapy rather than radiation. This younger group of patients had worse survival (P < 0.01) than the older age group. Other than young age, less than GTR and World Health Organization (WHO) II grade 3 histology were significant adverse risk factors for EFS in univariate and multivariate analyses. OS shared the same adverse risk factors except for histology in multivariate analysis, which was only of borderline significance (P = 0.05). Progression at the original tumor location, present in 89% of patients, was the major pattern of tumor recurrence. Adjuvant chemotherapy in the group older than 3 years or craniospinal radiation in M0 patients did not significantly change EFS. Conclusions: Adverse outcome in childhood intracranial ependymoma is related to age (3 years or younger), histology (grade 3), and degree of surgical resection (less than GTR). New approaches, particularly for local tumor control in younger patients, are needed to improve survival.
KW - Children
KW - Ependymoma
KW - Multivariate analysis
KW - Risk factors
KW - Survival
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U2 - 10.1097/00043426-199905000-00008
DO - 10.1097/00043426-199905000-00008
M3 - Article
C2 - 10363853
AN - SCOPUS:0033503453
VL - 21
SP - 203
EP - 211
JO - Journal of Pediatric Hematology/Oncology
JF - Journal of Pediatric Hematology/Oncology
SN - 1077-4114
IS - 3
ER -