Journal
CELLS
Volume 11, Issue 17, Pages -Publisher
MDPI
DOI: 10.3390/cells11172626
Keywords
epithelioid sarcoma; soft tissue sarcoma; SMARCB1; SWI; SNF; PRC2 (Polycomb Repressive Complex 2); tazemetostat
Categories
Funding
- Italian Ministry of Health
- Alleanza Contro il Cancro (ACC) Sarcoma WG
- Associazione Italiana Ricerca sul Cancro (AIRC ) [19975]
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This review summarizes the pathobiology of epithelioid sarcoma, including defects in chromatin remodeling and other signaling pathways, and their role as therapeutic vulnerabilities.
Epithelioid sarcoma (ES) is a very rare and aggressive mesenchymal tumor of unclear origin and uncertain lineage characterized by a prevalent epithelioid morphology. The only recurrent genetic alteration reported in ES as yet is the functional inactivation of SMARCB1 (SWI/SNF-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1), a key component of the SWI/SNF (SWItch/Sucrose Non-Fermentable) chromatin remodeling complexes. How SMARCB1 deficiency dictates the clinicopathological characteristics of ES and what other molecular defects concur to its malignant progression is still poorly understood. This review summarizes the recent findings about ES pathobiology, including defects in chromatin remodeling and other signaling pathways and their role as therapeutic vulnerabilities.
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