Ongoing Response in a Multiply Relapsed Metastatic Posterior Fossa Ependymoma A After Vorinostat and Concomitant Irradiation.
Document Type
Article
Publication Date
3-1-2022
Publication Title
Journal of Pediatric Hematology/Oncology
Abstract
Posterior fossa ependymomas A confer the worst prognosis among all subtypes. They demonstrate distinct epigenetic changes, which can be targeted with epigenetic modifiers like histone deacetylase inhibitors (Vorinostat). We describe a 3-year-old male diagnosed with a posterior fossa ependymoma who had a number of recurrences requiring multimodal therapy. Molecular analysis demonstrated a BCL-6 corepressor mutation, and methylation profiling matched with posterior fossa ependymomas A. He received craniospinal irradiation and focal boost with Vorinostat. Serial imaging after irradiation revealed a progressively decreasing tumor burden with nearly complete resolution of disease at 15 months. Histone deacetylase inhibitors demonstrate promise in treatment of carefully selected cases of ependymoma.
Volume
44
Issue
2
First Page
e576
Last Page
e579
Recommended Citation
Gorsi HS, Toll SA, Sood S, Miler S, Altinok D, Kumar-Sinha C, et al [Yankelevich M] Ongoing response in a multiply relapsed metastatic posterior fossa ependymoma A after vorinostat and concomitant irradiation. J Pediatr Hematol Oncol. 2022 Mar 1;44(2):e576-e579. doi: 10.1097/MPH.0000000000002175. PMID: 33930008.
DOI
10.1097/MPH.0000000000002175
ISSN
1536-3678
PubMed ID
33930008