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Purpose <p>The prognosis of intracranial ependymoma correlates to complete surgery followed by focal irradiation(RT).No standard therapies exist at relapse.</p> Methods <p>We analyzed relapsed patients between 2009 and 2023 both diagnosed in our institution as well as referred.</p> Results <p>Patients were 33 (females 12), median age 3.3 years. All treated according to AIEOP1st -2nd or SIOP 2nd protocol, i.e., operated and focally irradiated; some had chemotherapy (VEC or VEC/cisplatin + VCR) either after surgery(#13 for residual disease) or after radiotherapy(#10)0.25 tumors originated in posterior fossa,29 were grade 3,23 were tumor-free at RT. Relapses appeared at median 31 months after diagnosis: local in 22 cases, disseminated/combined in 11.21 tumors classified as PFA,8 as RELA/ZFTA.19 patients were again completely operated,16 received hypofractionated reirradiation at 8–16&#xa0;Gy in 2/4 fractions,6 standard re-RT at 18–54&#xa0;Gy,10 36&#xa0;Gy craniospinal irradiation for dissemination; one no treatment. We evaluated the first EFS, OS, and post-first relapse EFS/OS (EFS2/OS2). Median EFS, OS, EFS2 and OS2 were 31.1,66.4,13.6 and 31.5 months, respectively. EFS was positively associated to female sex, grade 3,complete resection at diagnosis/at relapse(also of single metastatic relapse). RELA/ZFTA molecular subgroup tumors affected positively on OS2. Supratentorial location and RELA/ZFTA showed an association that approached statistical significance on EFS2. Type of re-RT had no impact on analyses, with craniospinal radiotherapy adopted only for disseminated/combined relapse. In mutivariable analysis RELA/ZFTA subgroup and complete resection at relapse were associated with improved OS. 13/33 patients survived more than 7 years: 9 alive and 7 tumor-free.</p> Conclusions <p>Complete resection is a therapeutic resource also after relapse, ideal re-RT has to be found; outcome differed by molecular subgroup in our cohort.</p>

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Surgery and radiotherapy strategies at first relapse of pediatric intracranial ependymoma: an Italian retrospective study integrating molecular subgrouping

  • 期刊论文

  • 发表日期：

    2026年4月24日

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  • Maura Massimino,
  • Giorgio Carrabba,
  • Sabina Vennarini,
  • Emilia Pecori,
  • Paolo Ferroli,
  • Giuseppe Cinalli,
  • Veronica Biassoni,
  • Elisabetta Schiavello,
  • Olga Nigro,
  • Andrea Trezza,
  • Marco Schiariti,
  • Chiara Dossena,
  • Piergiorgio Modena,
  • Loris De Cecco,
  • Manila Antonelli,
  • Francesca Gianno,
  • Francesca Buttarelli,
  • Simone Minasi,
  • Alessandra Erbetta,
  • Luisa Chiapparini,
  • Silvia Esposito,
  • Arianna Trovò,
  • Valeria Barresi,
  • Carlo Giussani,
  • Francesco Barretta

摘要

Purpose

The prognosis of intracranial ependymoma correlates to complete surgery followed by focal irradiation(RT).No standard therapies exist at relapse.

Methods

We analyzed relapsed patients between 2009 and 2023 both diagnosed in our institution as well as referred.

Results

Patients were 33 (females 12), median age 3.3 years. All treated according to AIEOP1st -2nd or SIOP 2nd protocol, i.e., operated and focally irradiated; some had chemotherapy (VEC or VEC/cisplatin + VCR) either after surgery(#13 for residual disease) or after radiotherapy(#10)0.25 tumors originated in posterior fossa,29 were grade 3,23 were tumor-free at RT. Relapses appeared at median 31 months after diagnosis: local in 22 cases, disseminated/combined in 11.21 tumors classified as PFA,8 as RELA/ZFTA.19 patients were again completely operated,16 received hypofractionated reirradiation at 8–16 Gy in 2/4 fractions,6 standard re-RT at 18–54 Gy,10 36 Gy craniospinal irradiation for dissemination; one no treatment. We evaluated the first EFS, OS, and post-first relapse EFS/OS (EFS2/OS2). Median EFS, OS, EFS2 and OS2 were 31.1,66.4,13.6 and 31.5 months, respectively. EFS was positively associated to female sex, grade 3,complete resection at diagnosis/at relapse(also of single metastatic relapse). RELA/ZFTA molecular subgroup tumors affected positively on OS2. Supratentorial location and RELA/ZFTA showed an association that approached statistical significance on EFS2. Type of re-RT had no impact on analyses, with craniospinal radiotherapy adopted only for disseminated/combined relapse. In mutivariable analysis RELA/ZFTA subgroup and complete resection at relapse were associated with improved OS. 13/33 patients survived more than 7 years: 9 alive and 7 tumor-free.

Conclusions

Complete resection is a therapeutic resource also after relapse, ideal re-RT has to be found; outcome differed by molecular subgroup in our cohort.