| Title: |
EPEN-11. ONGOING RESPONSE IN A MULTIPLY RELAPSED METASTATIC POSTERIOR FOSSA EPENDYMOMA A AFTER VORINOSTAT AND CONCOMITANT IRRADIATION |
| Authors: |
Gorsi, Hamza S; Toll, Stephanie; Yankelevich, Maxim |
| Source: |
Neuro-Oncology ; volume 22, issue Supplement_3, page iii310-iii310 ; ISSN 1522-8517 1523-5866 |
| Publisher Information: |
Oxford University Press (OUP) |
| Publication Year: |
2020 |
| Description: |
BACKGROUND Among the nine molecular subgroups of ependymoma identified, posterior fossa ependymoma A (PF-EPN-A) confers the worst prognosis. These tumors often relapse despite aggressive resection and irradiation, resulting in limited therapeutic options. Although the genomic profile of PF-EPN-A does not typically show any recurrent alterations; they demonstrate distinct epigenetic changes which can be targeted with modulators such as histone deacetylase (HDAC) inhibitors. These inhibitors have shown efficacy in pre-clinical studies in both their anticancer and radio-sensitizing properties. CASE: We describe a male diagnosed with a posterior fossa ependymoma at 3 years of age. After initial therapy with resection and focal irradiation, he went on to have a number of recurrences requiring multimodal therapy. Most recently, he developed diffuse intraventricular and leptomeningeal disease not amenable to surgical intervention. Genetic evaluation demonstrated a BCOR mutation and methylation profile was consistent with PF-EPN-A. He received 23.4 Gray craniospinal irradiation with a 30.6 Gray boost to the nodular lesions. Vorinostat was given concomitantly for radio-sensitization in 2 week intervals for a total of 6 weeks. Serial imaging after irradiation revealed decreased tumor burden with almost complete resolution of disease at 15 months. Unfortunately, MRI at 18 months exhibited mild interval growth of 2 lesions. CONCLUSIONS To our knowledge, this is the first report of a clinical response in a pediatric patient with PF-EPN-A following irradiation administered concomitantly with vorinostat therapy. This response highlights the importance of further studies evaluating this combination therapy and its potential use in this population. |
| Document Type: |
article in journal/newspaper |
| Language: |
English |
| DOI: |
10.1093/neuonc/noaa222.151 |
| Availability: |
https://doi.org/10.1093/neuonc/noaa222.151; http://academic.oup.com/neuro-oncology/article-pdf/22/Supplement_3/iii310/34686692/noaa222.151.pdf |
| Rights: |
http://creativecommons.org/licenses/by-nc/4.0/ |
| Accession Number: |
edsbas.FD47CCA9 |
| Database: |
BASE |