Weighing ependymoma as an epigenetic disease
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TOPIC REVIEW
Weighing ependymoma as an epigenetic disease A. Stuckert1 · K. C. Bertrand1 · P. Wang2 · A. Smith3 · S. C. Mack1 Received: 7 June 2020 / Accepted: 15 June 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Introduction Ependymoma is the third most common malignant pediatric brain tumor. Although the biology that drives ependymoma is slowly being unraveled, the ability to translate these findings to clinical care remains an ongoing challenge. Epigenetic alterations appear to play a central role in the development of molecular classification of ependymoma. Methods We reviewed the published literature available describing genetic and epigenetic underpinnings of ependymoma that have been reported to date and have summarized the information regarding genetic drivers of ependymoma that may point us toward therapeutic strategies. Results Ependymoma is a molecularly heterogeneous disease which has now been divided into at least nine distinct molecular subtypes based on DNA methylation and gene expression profiling. DNA methylation has emerged as an effective tool for classification of brain tumors alongside histopathology and other molecular diagnostics. There have been large retrospective cohorts describing molecular subgroup identity as a powerful independent predictor of outcome. There is limited published data on prospective trials to date however this is forthcoming which will lead to molecular stratification in the next generation of clinical studies. Conclusion This is a review of recent advancements in our understanding of the epigenetic basis of ependymoma and discussion of how these findings reveal potential therapeutic opportunities. Keywords Ependymoma · Pediatric · Epigenetics · Cancer · Brain
Introduction Ependymoma is an aggressive and relentless disease that is one of the most common malignant brain tumors found in children. Brain tumors are the leading cause of cancer related death in pediatrics and despite decades of progress in many other tumor types, the mainstay of treatment for ependymoma patients remains maximal safe surgical resection and radiation therapy. No cytotoxic chemotherapy or targeted agents have been shown to have clear benefits in improving patient outcomes to date [1–5]. Although about * A. Smith [email protected] * S. C. Mack [email protected] 1
Baylor College of Medicine, Texas Children’s Cancer and Hematology Centers, Houston, TX, USA
2
Rice University, 6100 Main St, Houston, TX 77005, USA
3
Department of Pediatric Hematology‑Oncology, Arnold Palmer Hospital, Orlando, USA
75% of patients with ependymoma survive for over 5 years, most are left with neurologic sequelae of their treatment, which has a significant impact on their quality of life [6]. Ependymoma patients who relapse are often treated with surgery and re-irradiation. Effective and safe targeted therapies are desperately needed for treatment of ependymoma patients. Unfortunately, ependymomas harbor relatively low mutation rates, thus posing a challe
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