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

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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.

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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.

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Correspondence to Maura Massimino.

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The authors declare that they have no conflict of interest.

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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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Massimino, M., Barretta, F., Modena, P. et al. Pediatric intracranial ependymoma: correlating signs and symptoms at recurrence with outcome in the second prospective AIEOP protocol follow-up. J Neurooncol 140, 457–465 (2018). https://doi.org/10.1007/s11060-018-2974-6

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  • DOI: https://doi.org/10.1007/s11060-018-2974-6

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