Abstract

Abstract BACKGROUND Ependymoma is a rare disease in adult patients (pts) and there is no standard systemic treatment in recurrent disease after surgery and radiotherapy. We reviewed our cases of confirmed intracranial and spinal ependymomas and described experience of pts treated with systemic therapies at disease recurrence. MATERIAL AND METHODS We retrospectively collected data of pts with histopathologically confirmed intracranial and spinal ependymomas from Veneto Institute of Oncology IOV in Padua, Italy from April 2018 to April 2023 and identified a subgroup of pts that received at least one line of systemic therapy at disease recurrence. All ependymomas were classified according to WHO 2016. As local protocol, brain and spinal MRI was performed every 2-3 months or when clinically indicated. RANO criteria were used to neuroradiological response. Primary endpoints were disease control rate and progression-free survival. RESULTS A total of 36 adult pts with ependymoma were identified, 18 males, median age at diagnosis 43. Tumor locations were spine (n=6), infratentorial (n=6), supratentorial (n=24). The majority were grade 3 (n=15), the others grade 2 (n=11) and grade 1 ependymoma (n=10).9 selected pts received at least one line of systemic treatments at disease recurrence (6 female, 3 male, median age at diagnosis 42 years). The majority were grade 3 (n=7), the others grade 2 (n=2). All 9 pts had undergone surgery and adjuvant radiotherapy. In this subgroup, the median time to recurrence from diagnosis was 12.4 months (range 3-32). At disease recurrence surgery was re-performed in 3 pts. Median number of lines of systemic therapies was 2, with a range from 1 to 6. TMZ alone in 4 pts with a DCR 40% and mPFS 4ms; TMZ+ cisplatin in 2 pts (DCR 50%, mPFS 4ms); TMZ+lapatinib in 3 pts (DCR 33%, mPFS 3.5ms); TMZ+bevacizumab in 1 pt (DCR 0%, mPFS 2ms); bevacizumab alone in 2 pts (DCR 0%, mPFS 2ms); bevacizumab+fotemustine in 1 pts (DCR 100%, mPFS 14ms), fotemustine alone in 2 pts (DCR 50%, mPFS 7.5ms); carboplatin 1 pts (DCR 0%, mPFS 2ms). At the data cut-off (April 2023), median overall survival from the date of diagnosis was 18.5 months (range 2-50). CONCLUSION The management of recurrent ependymoma in adults remains a therapeutic challenge. Our experience reflects heterogeneity regarding systemic treatments for patients with more aggressive ependymomas. Even if it was a small population, TMZ plus cisplatin ,fotemustine alone or in association with bevacizumab might represent promising treatments. However, other larger and prospective studies are needed.

Full Text
Published version (Free)

Talk to us

Join us for a 30 min session where you can share your feedback and ask us any queries you have

Schedule a call