Metastatic ependymoma: A multi-institutional retrospective analysis of prognostic factors

Stergios Zacharoulis, Lingyun Ji, Ian F. Pollack, Patricia Duffner, Russel Geyer, Jacques Grill, Steven Schild, Tang Her Jaing, Maura Massimino, Jonathan Finlay, Richard Sposto

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Abstract

Purpose. Metastatic ependymoma is exceedingly rare at diagnosis with variable prognosis reported in the literature. The purpose of this study was to identify prognostic factors in children with metastatic ependymoma. Procedure. Data regarding diagnosis, treatment and follow-up for 40 patients from eight institutional cohorts were collected. Results. Twenty-nine (72%) patients were less than 36 months of age at the time of diagnosis, 28% were females, and 90% of the patients had posterior fossa tumors. Gross total resection (GTR) of the primary tumor was achieved in 16 patients (40%). Adjuvant therapy was variable and included craniospinal irradiation (CSRT), chemotherapy, and chemotherapy with focal irradiation. The 5-year event free survival (EFS) and overall survival (OS) from the time of diagnosis were 29% (±7%) and 43% (±8%), respectively. Age at diagnosis was associated significantly with both EFS and OS (P < 0.001 for EFS, and P = 0.01 for OS). Patients who were 24-35 months of age at diagnosis had a 5-year EFS of 66% and a 5-year OS of 73%; both survival rates were superior to those of patients younger than 24 months of age or older than 36 months. Patients with GTR achieved a 5-year EFS of 35% and OS of 59%, compared to a 5-year EFS of 25% and OS of 32% for patients who did not achieve GTR (P = 0.12 for EFS, P = 0.03 for OS). Conclusions. GTR should be attempted in patients with metastatic ependymoma. A subgroup of patients between 24 and 35 months might have a favorable outcome.

Original languageEnglish (US)
Pages (from-to)231-235
Number of pages5
JournalPediatric Blood and Cancer
Volume50
Issue number2
DOIs
StatePublished - Feb 2008

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Ependymoma
Disease-Free Survival
Survival
Craniospinal Irradiation
Infratentorial Neoplasms
Drug Therapy
Survival Rate

Keywords

  • Metastatic ependymoma
  • Prognosis

ASJC Scopus subject areas

  • Cancer Research
  • Pediatrics, Perinatology, and Child Health
  • Hematology

Cite this

Zacharoulis, S., Ji, L., Pollack, I. F., Duffner, P., Geyer, R., Grill, J., ... Sposto, R. (2008). Metastatic ependymoma: A multi-institutional retrospective analysis of prognostic factors. Pediatric Blood and Cancer, 50(2), 231-235. https://doi.org/10.1002/pbc.21276

Metastatic ependymoma : A multi-institutional retrospective analysis of prognostic factors. / Zacharoulis, Stergios; Ji, Lingyun; Pollack, Ian F.; Duffner, Patricia; Geyer, Russel; Grill, Jacques; Schild, Steven; Jaing, Tang Her; Massimino, Maura; Finlay, Jonathan; Sposto, Richard.

In: Pediatric Blood and Cancer, Vol. 50, No. 2, 02.2008, p. 231-235.

Research output: Contribution to journalArticle

Zacharoulis, S, Ji, L, Pollack, IF, Duffner, P, Geyer, R, Grill, J, Schild, S, Jaing, TH, Massimino, M, Finlay, J & Sposto, R 2008, 'Metastatic ependymoma: A multi-institutional retrospective analysis of prognostic factors', Pediatric Blood and Cancer, vol. 50, no. 2, pp. 231-235. https://doi.org/10.1002/pbc.21276
Zacharoulis, Stergios ; Ji, Lingyun ; Pollack, Ian F. ; Duffner, Patricia ; Geyer, Russel ; Grill, Jacques ; Schild, Steven ; Jaing, Tang Her ; Massimino, Maura ; Finlay, Jonathan ; Sposto, Richard. / Metastatic ependymoma : A multi-institutional retrospective analysis of prognostic factors. In: Pediatric Blood and Cancer. 2008 ; Vol. 50, No. 2. pp. 231-235.
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abstract = "Purpose. Metastatic ependymoma is exceedingly rare at diagnosis with variable prognosis reported in the literature. The purpose of this study was to identify prognostic factors in children with metastatic ependymoma. Procedure. Data regarding diagnosis, treatment and follow-up for 40 patients from eight institutional cohorts were collected. Results. Twenty-nine (72{\%}) patients were less than 36 months of age at the time of diagnosis, 28{\%} were females, and 90{\%} of the patients had posterior fossa tumors. Gross total resection (GTR) of the primary tumor was achieved in 16 patients (40{\%}). Adjuvant therapy was variable and included craniospinal irradiation (CSRT), chemotherapy, and chemotherapy with focal irradiation. The 5-year event free survival (EFS) and overall survival (OS) from the time of diagnosis were 29{\%} (±7{\%}) and 43{\%} (±8{\%}), respectively. Age at diagnosis was associated significantly with both EFS and OS (P < 0.001 for EFS, and P = 0.01 for OS). Patients who were 24-35 months of age at diagnosis had a 5-year EFS of 66{\%} and a 5-year OS of 73{\%}; both survival rates were superior to those of patients younger than 24 months of age or older than 36 months. Patients with GTR achieved a 5-year EFS of 35{\%} and OS of 59{\%}, compared to a 5-year EFS of 25{\%} and OS of 32{\%} for patients who did not achieve GTR (P = 0.12 for EFS, P = 0.03 for OS). Conclusions. GTR should be attempted in patients with metastatic ependymoma. A subgroup of patients between 24 and 35 months might have a favorable outcome.",
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T2 - A multi-institutional retrospective analysis of prognostic factors

AU - Zacharoulis, Stergios

AU - Ji, Lingyun

AU - Pollack, Ian F.

AU - Duffner, Patricia

AU - Geyer, Russel

AU - Grill, Jacques

AU - Schild, Steven

AU - Jaing, Tang Her

AU - Massimino, Maura

AU - Finlay, Jonathan

AU - Sposto, Richard

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N2 - Purpose. Metastatic ependymoma is exceedingly rare at diagnosis with variable prognosis reported in the literature. The purpose of this study was to identify prognostic factors in children with metastatic ependymoma. Procedure. Data regarding diagnosis, treatment and follow-up for 40 patients from eight institutional cohorts were collected. Results. Twenty-nine (72%) patients were less than 36 months of age at the time of diagnosis, 28% were females, and 90% of the patients had posterior fossa tumors. Gross total resection (GTR) of the primary tumor was achieved in 16 patients (40%). Adjuvant therapy was variable and included craniospinal irradiation (CSRT), chemotherapy, and chemotherapy with focal irradiation. The 5-year event free survival (EFS) and overall survival (OS) from the time of diagnosis were 29% (±7%) and 43% (±8%), respectively. Age at diagnosis was associated significantly with both EFS and OS (P < 0.001 for EFS, and P = 0.01 for OS). Patients who were 24-35 months of age at diagnosis had a 5-year EFS of 66% and a 5-year OS of 73%; both survival rates were superior to those of patients younger than 24 months of age or older than 36 months. Patients with GTR achieved a 5-year EFS of 35% and OS of 59%, compared to a 5-year EFS of 25% and OS of 32% for patients who did not achieve GTR (P = 0.12 for EFS, P = 0.03 for OS). Conclusions. GTR should be attempted in patients with metastatic ependymoma. A subgroup of patients between 24 and 35 months might have a favorable outcome.

AB - Purpose. Metastatic ependymoma is exceedingly rare at diagnosis with variable prognosis reported in the literature. The purpose of this study was to identify prognostic factors in children with metastatic ependymoma. Procedure. Data regarding diagnosis, treatment and follow-up for 40 patients from eight institutional cohorts were collected. Results. Twenty-nine (72%) patients were less than 36 months of age at the time of diagnosis, 28% were females, and 90% of the patients had posterior fossa tumors. Gross total resection (GTR) of the primary tumor was achieved in 16 patients (40%). Adjuvant therapy was variable and included craniospinal irradiation (CSRT), chemotherapy, and chemotherapy with focal irradiation. The 5-year event free survival (EFS) and overall survival (OS) from the time of diagnosis were 29% (±7%) and 43% (±8%), respectively. Age at diagnosis was associated significantly with both EFS and OS (P < 0.001 for EFS, and P = 0.01 for OS). Patients who were 24-35 months of age at diagnosis had a 5-year EFS of 66% and a 5-year OS of 73%; both survival rates were superior to those of patients younger than 24 months of age or older than 36 months. Patients with GTR achieved a 5-year EFS of 35% and OS of 59%, compared to a 5-year EFS of 25% and OS of 32% for patients who did not achieve GTR (P = 0.12 for EFS, P = 0.03 for OS). Conclusions. GTR should be attempted in patients with metastatic ependymoma. A subgroup of patients between 24 and 35 months might have a favorable outcome.

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KW - Prognosis

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