Pediatric intracranial ependymoma: correlating signs and symptoms at recurrence with outcome in the second prospective AIEOP protocol follow-up
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.
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.
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.
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.
KeywordsChildhood ependymoma Relapse Surveillance Follow-up Re-irradiation
We thank all the neurosurgery, radiotherapy, and pediatric departments, all the families and kids, and all the data managers involved in this study.
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.
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 was obtained from all the individuals (or their parents/tutors) who participated in the study.
- 1.Bouffet E, Doz F, Demaille MC, Tron P, Roche H, Plantaz D, Thyss A, Stephan JL, Lejars O, Sariban E, Buclon M, Zücker JM, Brunat-Mentigny M, Bernard JL, Gentet JC (1998) Improving survival in recurrent medulloblastoma: earlier detection, better treatment or still an impasse? Br J Cancer 77:1321–1326CrossRefGoogle Scholar
- 3.Spreafico F, Gandola L, Marchianò A, Simonetti F, Poggi G, Adduci A, Clerici CA, Luksch R, Biassoni V, Meazza C, Catania S, Terenziani M, Musumeci R, Fossati-Bellani F, Massimino M (2008) Brain magnetic resonance imaging after high-dose chemotherapy and radiotherapy for childhood brain tumors. Int J Radiat Oncol Biol Phys 70:1011–1019CrossRefGoogle Scholar
- 5.Massimino M, Miceli R, Giangaspero F, Boschetti L, Modena P, Antonelli M, Ferroli P, Bertin D, Pecori E, Valentini L, Biassoni V, Garrè ML, Schiavello E, Sardi I, Cama A, Viscardi E, Scarzello G, Scoccianti S, Mascarin M, Quaglietta L, Cinalli G, Diletto B, Genitori L, Peretta P, Mussano A, Buccoliero A, Calareso G, Barra S, Mastronuzzi A, Giussani C, Marras CE, Balter R, Bertolini P, Giombelli E, La Spina M, Buttarelli FR, Pollo B, Gandola L (2016) Final results of the second prospective AIEOP protocol for pediatric intracranial ependymoma. Neuro Oncol 18:1451–1460CrossRefGoogle Scholar
- 23.Bouffet E, Hawkins CE, Ballourah W, Taylor MD, Bartels UK, Schoenhoff N, Tsangaris E, Huang A, Kulkarni A, Mabbot DJ, Laperriere N, Tabori U (2012) Survival benefit for pediatric patients with recurrent ependymoma treated with reirradiation. Int J Radiat Oncol Biol Phys 83:1541–1548CrossRefGoogle Scholar