Treatment and outcome of intracranial ependymoma after first relapse in the 2nd AIEOP protocol.

Abstract

BACKGROUND:

More than 40% of patients with intracranial ependymoma need a salvage treatment within 5 years after diagnosis, and no standard treatment is available as yet. We report the outcome after first relapse of 64 patients treated within the 2nd AIEOP protocol.

METHODS:

We considered relapse sites and treatments, that is, various combinations of complete/incomplete surgery, if followed by standard or hypofractionated radiotherapy (RT) ± chemotherapy (CT). Molecular analyses were available for 38/64 samples obtained at first diagnosis. Of the 64 cases, 55 were suitable for subsequent analyses.

RESULTS:

The median follow-up was 147 months after diagnosis, 84 months after first relapse, 5-year EFS/OS were 26.2%/30.8% (median EFS/OS 13/32 months) after relapse. For patients with a local relapse (LR), the 5-year cumulative incidence of second LRs was 51.6%, with a 5-year event-specific probability of being LR-free of 40.0%. Tumor site/grade, need for shunting, age above/below 3 years, molecular subgroup at diagnosis, had no influence on outcomes. Due to variation in the RT dose/fractionation used and the subgroup sizes, it was not possible to assess the impact of the different RT modalities. Multivariable analyses identified completion of surgery, the absence of symptoms at relapse, and female sex as prognostically favorable. Tumors with a 1q gain carried a higher cumulative incidence of dissemination after first relapse.

CONCLUSIONS:

Survival after recurrence was significantly influenced by symptoms and completeness of surgery. Only a homogeneous protocol with well-posed, randomized questions could clarify the numerous issues, orient salvage treatment, and ameliorate prognosis for this group of patients.

• Authors: Maura Massimino, Francesco Barretta, Piergiorgio Modena, Pascal Johann, Paolo Ferroli, Manila Antonelli, Lorenza Gandola, Maria Luisa Garrè, Daniele Bertin, Angela Mastronuzzi, Maurizio Mascarin, Lucia Quaglietta, Elisabetta Viscardi, Iacopo Sardi, Antonio Ruggiero, Luna Boschetti, Marzia Giagnacovo, Veronica Biassoni, Elisabetta Schiavello, Luisa Chiapparini, Alessandra Erbetta, Anna Mussano, Carlo Giussani, Rosa Maria Mura, Salvina Barra, Giovanni Scarzello, Giuseppe Scimone, Andrea Carai, Felice Giangaspero, Francesca Romana Buttarelli
• Journal: Neuro-oncology (Neuro Oncol) Vol. 24 Issue 3 Pg. 467-479 (03 12 2022) ISSN: 1523-5866 [Electronic] England
• PMID: 34605902 (Publication Type: Journal Article)
• Copyright: © The Author(s) 2021. Published by Oxford University Press on behalf of the Society for Neuro-Oncology. All rights reserved. For permissions, please e-mail: [email protected].

Topics

• Brain Neoplasms (pathology)
• Child, Preschool
• Ependymoma (pathology)
• Female
• Humans
• Neoplasm Recurrence, Local (therapy)
• Prognosis
• Treatment Outcome