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Journal of Neuro-Oncology

, Volume 140, Issue 2, pp 457–465 | Cite as

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

  • Maura Massimino
  • Francesco Barretta
  • Piergiorgio Modena
  • Felice Giangaspero
  • Luisa Chiapparini
  • Alessandra Erbetta
  • Luna Boschetti
  • Manila Antonelli
  • Paolo Ferroli
  • Daniele Bertin
  • Emilia Pecori
  • Veronica Biassoni
  • Maria Luisa Garrè
  • Elisabetta Schiavello
  • Iacopo Sardi
  • Elisabetta Viscardi
  • Giovanni Scarzello
  • Maurizio Mascarin
  • Lucia Quaglietta
  • Giuseppe Cinalli
  • Lorenzo Genitori
  • Paola Peretta
  • Anna Mussano
  • Salvina Barra
  • Angela Mastronuzzi
  • Carlo Giussani
  • Carlo Efisio Marras
  • Rita Balter
  • Patrizia Bertolini
  • Assunta Tornesello
  • Milena La Spina
  • Francesca Romana Buttarelli
  • Antonio Ruggiero
  • Massimo Caldarelli
  • Geraldina Poggi
  • Lorenza Gandola
Clinical Study

Abstract

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.

Keywords

Childhood ependymoma Relapse Surveillance Follow-up Re-irradiation 

Notes

Acknowledgements

We thank all the neurosurgery, radiotherapy, and pediatric departments, all the families and kids, and all the data managers involved in this study.

Funding

The Associazione Bianca Garavaglia Onlus, Busto Arsizio (VA) conv. 2015–2017; AIRC (Associazione Italiana per la Ricerca sul Cancro) interreg. project 2005–2010; Associazione Bimbo Tu, Bologna 2017, Italy.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures were in accordance with the ethical standards of the institutional and/or national research committee, and with the 1964 Helsinki Declaration and its later amendments, or comparable ethical standards.

Informed consent

Informed consent was obtained from all the individuals (or their parents/tutors) who participated in the study.

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Maura Massimino
    • 1
  • Francesco Barretta
    • 2
  • Piergiorgio Modena
    • 3
  • Felice Giangaspero
    • 4
    • 5
  • Luisa Chiapparini
    • 6
  • Alessandra Erbetta
    • 6
  • Luna Boschetti
    • 1
  • Manila Antonelli
    • 4
  • Paolo Ferroli
    • 7
  • Daniele Bertin
    • 8
  • Emilia Pecori
    • 31
  • Veronica Biassoni
    • 1
  • Maria Luisa Garrè
    • 9
  • Elisabetta Schiavello
    • 1
  • Iacopo Sardi
    • 10
  • Elisabetta Viscardi
    • 11
  • Giovanni Scarzello
    • 12
  • Maurizio Mascarin
    • 13
  • Lucia Quaglietta
    • 14
  • Giuseppe Cinalli
    • 15
  • Lorenzo Genitori
    • 16
  • Paola Peretta
    • 17
  • Anna Mussano
    • 18
  • Salvina Barra
    • 19
  • Angela Mastronuzzi
    • 20
  • Carlo Giussani
    • 21
  • Carlo Efisio Marras
    • 22
  • Rita Balter
    • 23
  • Patrizia Bertolini
    • 24
  • Assunta Tornesello
    • 25
  • Milena La Spina
    • 26
  • Francesca Romana Buttarelli
    • 27
  • Antonio Ruggiero
    • 28
  • Massimo Caldarelli
    • 29
  • Geraldina Poggi
    • 30
  • Lorenza Gandola
    • 31
  1. 1.Pediatric Unit (MM, LB, VB, ES)Fondazione IRCCS Istituto Nazionale dei TumoriMilanItaly
  2. 2.Clinical Epidemiology and Trial Organization Unit (FB)Fondazione IRCCS Istituto Nazionale dei TumoriMilanItaly
  3. 3.Laboratory of Genetics, Pathology Unit (PM)S. Anna General HospitalComoItaly
  4. 4.Department of Radiological, Oncological and Anatomo-Pathological Sciences (FG, MA)Sapienza UniversityRomeItaly
  5. 5.IRCCS Neuromed (FG)PozzuoliItaly
  6. 6.Neuroradiology (LC, AE)Fondazione IRCCS Istituto Neurologico Carlo BestaMilanItaly
  7. 7.Neurosurgery Department (PF)Fondazione IRCCS Istituto Neurologico Carlo BestaMilanItaly
  8. 8.Pediatric Onco-Hematology (DB)A.O.U. Città della Salute e della ScienzaTurinItaly
  9. 9.Neurosurgery Unit (MLG)Istituto Giannina GasliniGenoaItaly
  10. 10.Neuro-oncology (IS)Ospedale Pediatrico MeyerFlorenceItaly
  11. 11.Pediatric Oncology Unit, (EV)Padova UniversityPaduaItaly
  12. 12.Radiotherapy Department (GS)IOV-IRCCSPaduaItaly
  13. 13.Pediatric Radiotherapy (MaM), CROAvianoItaly
  14. 14.Pediatric Oncology (LQ)Ospedale Santobono-PausilliponNaplesItaly
  15. 15.Neurosurgery Unit (GC)Ospedale Santobono-PausilliponNaplesItaly
  16. 16.Neurosurgery Unit (LG)Ospedale Pediatrico MeyerFlorenceItaly
  17. 17.Pediatric Neurosurgery Unit (PP)A.O.U. Città della Salute e della ScienzaTurinItaly
  18. 18.Radiotherapy (AM)A.O.U. Città della Salute e della ScienzaTurinItaly
  19. 19.Department of Radiation Oncology (SB)IRCCS San Martino ISTGenoaItaly
  20. 20.Pediatric Hematology and Oncology Unit (AnM)Ospedale Pediatrico Bambino GesùRomeItaly
  21. 21.Neurosurgery Unit (CG), University of Milano-BicoccaOspedale San GerardoMonzaItaly
  22. 22.Neurosurgery Unit (CEM)Ospedale Pediatrico Bambino GesùRomeItaly
  23. 23.Pediatric Oncology Unit (RB)Ospedale della Donna e del BambinoVeronaItaly
  24. 24.Pediatric Oncology Unit (PB)University Hospital of ParmaParmaItaly
  25. 25.Pediatric Oncology Unit (AT)Ospedale Vito FazziLecceItaly
  26. 26.Unit of Paediatric Haematology and Oncology (MLS), Hospital PoliclinicoUniversity of CataniaCataniaItaly
  27. 27.Neurological Science (FRB)Sapienza UniversityRomeItaly
  28. 28.Pediatric Oncology (AR)Fondazione Policlinico Universitario Agostino GemelliRomeItaly
  29. 29.Neurosurgery (MC)Fondazione Policlinico Universitario Agostino GemelliRomeItaly
  30. 30.Acquired Brain Lesions Unit (GP)IRCCS Eugenio MedeaBosisio PariniItaly
  31. 31.Pediatric Radiotherapy Unit (LG, EP)Fondazione IRCCS Istituto Nazionale dei TumoriMilanItaly

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