Rhabdomyosarcoma (RMS) is the most prevalent soft tissue sarcoma in children, and despite advances in multimodal therapy, progress in improving the survival of high-risk patients has been limited. Increasing evidence indicates that epigenetic dysregulation contributes to RMS pathogenesis and therapeutic resistance, particularly through aberrant activity of histone deacetylases (HDACs). HDAC inhibitors (HDACi) have shown promise in preclinical RMS models, showing enhancing of the efficacy of standard chemotherapies and radiotherapy. This mini-review summarizes recent studies exploring HDAC inhibition in combination with first-line therapies, examines the mechanistic basis for therapeutic synergy, and discusses opportunities and challenges in translating HDACi-based combinations to the clinic. By integrating mechanistic insights with translational evidence, this review outlines current progress and proposes future directions for development of HDACi-enhanced treatment strategies for this aggressive pediatric malignancy.

Macrì, E., Attili, M., Locatelli, F., Marampon, F., Pomella, S. (2026). From bench to bedside: combining HDAC inhibitors with standard therapies in rhabdomyosarcoma treatment. FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY, 14 [10.3389/fcell.2026.1774090].

From bench to bedside: combining HDAC inhibitors with standard therapies in rhabdomyosarcoma treatment

Silvia Pomella
2026-01-01

Abstract

Rhabdomyosarcoma (RMS) is the most prevalent soft tissue sarcoma in children, and despite advances in multimodal therapy, progress in improving the survival of high-risk patients has been limited. Increasing evidence indicates that epigenetic dysregulation contributes to RMS pathogenesis and therapeutic resistance, particularly through aberrant activity of histone deacetylases (HDACs). HDAC inhibitors (HDACi) have shown promise in preclinical RMS models, showing enhancing of the efficacy of standard chemotherapies and radiotherapy. This mini-review summarizes recent studies exploring HDAC inhibition in combination with first-line therapies, examines the mechanistic basis for therapeutic synergy, and discusses opportunities and challenges in translating HDACi-based combinations to the clinic. By integrating mechanistic insights with translational evidence, this review outlines current progress and proposes future directions for development of HDACi-enhanced treatment strategies for this aggressive pediatric malignancy.
2026
Pubblicato
Rilevanza internazionale
Review
Esperti anonimi
Settore MED/05
Settore MEDS-02/B - Patologia clinica
English
chemo-radiotherapy sensitization;
epigenetic targeting;
HDAC inhibitor (histone deacetylase inhibitor);
rhabdomyosarcoma;
translational oncology
Macrì, E., Attili, M., Locatelli, F., Marampon, F., Pomella, S. (2026). From bench to bedside: combining HDAC inhibitors with standard therapies in rhabdomyosarcoma treatment. FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY, 14 [10.3389/fcell.2026.1774090].
Macrì, E; Attili, M; Locatelli, F; Marampon, F; Pomella, S
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2108/462563
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