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Case Reports
. 2025 Aug 1;47(6):e269-e272.
doi: 10.1097/MPH.0000000000003080. Epub 2025 Jul 1.

Adjuvant Etoposide for Very High-risk PFA Ependymoma: A Case Report

Jacob Silverman  1 , Melissa Mégalli  2 , Emilie Giguère  2 , Sarah Elbaz  2 , Melanie Letourneau  3 , Catherine Achim  4 , Cynthia Hawkins  5   6 , William Legallou  7 , Panagiota Giannakouros  7 , Sébastien Perreault  8 , Geneviève Legault  9 , Eric Bouffet  10 , Nada Jabado  9 , Hallie Coltin  11 , Louis Crevier  12 , Samuele Renzi  7
Affiliations
Case Reports

Adjuvant Etoposide for Very High-risk PFA Ependymoma: A Case Report

Jacob Silverman et al. J Pediatr Hematol Oncol. .

Abstract

Background: Ependymomas of the posterior fossa type A (PF-A) with a combined chromosome 1q gain and 6q loss are associated with an extremely high risk of recurrence and a very poor outcome.

Observations: We report the case of a 4-year-old girl who received adjuvant oral etoposide for 1 year after conventional treatment (surgery and focal radiation). The patient remains in clinical and radiologic remission 2.5 years post-diagnosis.

Conclusions: This approach of using oral etoposide could be considered in ultra-high-risk 6q loss PF-A ependymoma to try and decrease the risk of relapse, awaiting further evaluation in a clinical trial.

Keywords: 1q gain; 6q loss; ependymoma; etoposide; hydrocephaly; intracranial hypertension; proton therapy.

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Conflict of interest statement

The authors declare no conflict of interest..

References

    1. Baroni LV, Sundaresan L, Heled A, et al. Ultra high-risk PFA ependymoma is characterized by loss of chromosome 6q. Neuro Oncol. 2021;23:1360–1370.
    1. Merchant TE, Bendel AE, Sabin ND, et al. Conformal radiation therapy for pediatric ependymoma, chemotherapy for incompletely resected ependymoma, and observation for completely resected, supratentorial ependymoma. J Clin Oncol. 2019;37:974–983.
    1. Donson AM, Bertrand KC, Riemondy KA, et al. Significant increase of high-risk chromosome 1q gain and 6q loss at recurrence in posterior fossa group A ependymoma: a multicenter study. Neuro Oncol. 2023;25:1854–1867.
    1. Crotty E, Gust J. Pediatric ependymoma: new perspectives on older trials. Neuro Oncol. 2022;24:949–950.
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