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Ongoing Response in a Multiply Relapsed Metastatic Posterior Fossa Ependymoma A After Vorinostat and Concomitant Irradiation

Authors :
Hamza S. Gorsi
Steven Miler
Sandeep Sood
Deniz Altinok
Rajen Mody
Maxim Yankelevich
Stephanie A. Toll
Chandan Kumar-Sinha
Source :
Journal of Pediatric Hematology/Oncology. 44:e576-e579
Publication Year :
2021
Publisher :
Ovid Technologies (Wolters Kluwer Health), 2021.

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.

Details

ISSN :
10774114
Volume :
44
Database :
OpenAIRE
Journal :
Journal of Pediatric Hematology/Oncology
Accession number :
edsair.doi.dedup.....86f1ba16b8f5883eced9a3f9f1690bfc