Treatment implications of posterior fossa ependymoma subgroups.

Vijay Ramaswamy,Michael D Taylor

doi:10.1186/s40880-016-0155-6

Vijay Ramaswamy, Michael D Taylor

Open Access

https://doi.org/10.1186/s40880-016-0155-6

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Journal: Cancer Communications	Publication Date: Nov 15, 2016
Citations: 23	License type: cc-by

Affiliation: Hospital for Sick Children

Abstract

Posterior fossa ependymoma comprises two distinct molecular entities, ependymoma_posterior fossa A (EPN_PFA) and ependymoma_posterior fossa B (EPN_PFB), with differentiable gene expression profiles. As yet, the response of the two entities to treatment is unclear. To determine the relationship between the two molecular subgroups of posterior fossa ependymoma and treatment, we studied a cohort of 820 patients with molecularly profiled, clinically annotated posterior fossa ependymomas. We found that the strongest predictor of poor outcome in patients with posterior fossa ependymoma across the entire age spectrum was molecular subgroup EPN_PFA, which was recently reported in the paper entitled “Therapeutic impact of cytoreductive surgery and irradiation of posterior fossa ependymoma in the molecular era: a retrospective multicohort analysis” in the Journal of Clinical Oncology. Patients with incompletely resected EPN_PFA tumors had a very poor outcome despite receiving adjuvant radiation therapy, whereas a substantial proportion of patients with EPN_PFB tumors can be cured with surgery alone.

Highlights

Posterior fossa ependymoma comprises two distinct molecular entities, ependymoma_posterior fossa A (EPN_PFA) and ependymoma_posterior fossa B (EPN_PFB), with differentiable gene expression profiles
We showed that posterior fossa ependymoma comprises two distinct molecular entities called ependymoma_posterior fossa A (EPN_PFA) and ependymoma_posterior fossa
Using a multivariable model incorporating age, extent of surgical resection, postoperative first-line radiotherapy, adjuvant chemotherapy, and molecular subgroup, we found that the strongest predictor of poor outcome was EPN_PFA subgroup, with a hazard ratio of 2.14 (95% confidence interval [CI] 1.31–3.49) for progression and 4.3 for death

Summary

Introduction

Posterior fossa ependymoma comprises two distinct molecular entities, ependymoma_posterior fossa A (EPN_PFA) and ependymoma_posterior fossa B (EPN_PFB), with differentiable gene expression profiles. Current therapy for posterior fossa ependymoma in children older than 1 year consists of maximal safe surgical resection followed by 54–59 Gy of external beam irradiation to the tumor bed [15]. Using a multivariable model incorporating age, extent of surgical resection, postoperative first-line radiotherapy, adjuvant chemotherapy, and molecular subgroup, we found that the strongest predictor of poor outcome was EPN_PFA subgroup, with a hazard ratio of 2.14 (95% confidence interval [CI] 1.31–3.49) for progression and 4.3 (95% CI 1.88–9.87) for death.

Results

Conclusion