Pediatric intracranial ependymoma: correlating signs and symptoms at recurrence with outcome in the second prospective AIEOP protocol follow-up

M. Massimino, F. Barretta, P. Modena, F. Giangaspero, L. Chiapparini, A. Erbetta, L. Boschetti, M. Antonelli, P. Ferroli, D. Bertin, E. Pecori, V. Biassoni, M.L. Garrè, E. Schiavello, I. Sardi, E. Viscardi, G. Scarzello, M. Mascarin, L. Quaglietta, G. CinalliL. Genitori, P. Peretta, A. Mussano, S. Barra, A. Mastronuzzi, C. Giussani, C.E. Marras, R. Balter, P. Bertolini, A. Tornesello, M. La Spina, F.R. Buttarelli, A. Ruggiero, M. Caldarelli, G. Poggi, L. Gandola

Research output: Contribution to journalArticlepeer-review


Purpose: The aims of patients’ radiological surveillance are to: ascertain relapse; apply second-line therapy; accrue patients in phase 1/2 protocols if second-line therapy is not standardized/curative; and assess/treat iatrogenic effects. To lessen the emotional and socioeconomic burdens for patients and families, we ideally need to establish whether scheduled radiological surveillance gives patients a better outcome than waiting for symptoms and signs to appear. Methods: We analyzed a prospective series of 160 newly-diagnosed and treated pediatric/adolescent patients with intracranial ependymoma, comparing patients with recurrent disease identified on scheduled MRI (the RECPT group; 34 cases) with those showing signs/symptoms of recurrent disease (the SYMPPT group; 16 cases). The median follow-up was 67 months. Results: No significant differences emerged between the two groups in terms of gender, age, tumor grade/site, shunting, residual disease, or type of relapse (local, distant, or concomitant). The time to relapse (median 19 months; range 5–104) and the MRI follow-up intervals did not differ between the SYMPPT and RECPT groups. The presence of signs/symptoms was an unfavorable factor for overall survival (OS) after recurrence (5-year OS: 8% vs. 37%, p = 0.001). On multivariable analysis, an adjusted model confirmed a significantly worse OS in the SYMPPT than in the RECPT patients. Conclusions: Symptomatic relapses carried a significantly worse survival for ependymoma patients than recurrences detected by MRI alone. It would therefore be desirable to identify recurrences before symptoms develop. Radiological follow-up should be retained in ependymoma patient surveillance because there is a chance of salvage treatment for relapses found on MRI. © 2018, Springer Science+Business Media, LLC, part of Springer Nature.
Original languageEnglish
Pages (from-to)457-465
Number of pages9
JournalJournal of Neuro-Oncology
Issue number2
Publication statusPublished - 2018


  • Childhood ependymoma
  • Follow-up
  • Re-irradiation
  • Relapse
  • Surveillance
  • cyclophosphamide
  • etoposide
  • vincristine
  • adolescent
  • age
  • Article
  • brain cancer
  • brain surgery
  • cancer grading
  • cancer localization
  • cancer radiotherapy
  • cancer recurrence
  • cancer surgery
  • cancer survival
  • child
  • childhood cancer
  • clinical feature
  • clinical outcome
  • clinical protocol
  • clinical trial
  • cohort analysis
  • controlled study
  • ependymoma
  • female
  • follow up
  • gender
  • human
  • human tissue
  • major clinical study
  • male
  • minimal residual disease
  • nuclear magnetic resonance imaging
  • outcome assessment
  • overall survival
  • pediatric intracranial ependymoma
  • prospective study
  • retrospective study
  • survival rate
  • survival time
  • symptom
  • brain tumor
  • mortality
  • preschool child
  • prognosis
  • tumor recurrence
  • Adolescent
  • Brain Neoplasms
  • Child
  • Child, Preschool
  • Clinical Protocols
  • Ependymoma
  • Female
  • Follow-Up Studies
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Neoplasm Recurrence, Local
  • Prognosis
  • Prospective Studies


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