Ewing‐like sarcoma: An emerging family of round cell sarcomas
Journal of Cellular Physiology
Published online on September 26, 2018
Abstract
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- "\nAbstract\nEwing‐like sarcomas are an emerging subgroup of small round blue cell
sarcomas that share various degrees of morphological, immunohistochemical, molecular,
and clinical similarity with Ewing sarcoma. Despite these similarities, Ewing‐like
sarcomas lack the pathognomonic molecular hallmark of Ewing sarcoma: A translocation
between a gene of the RNA‐binding TET family (EWSR1 or \nFUS) with a gene of the
ETS‐transcription family (\nFLI1, \nERG, \nETV1, \nETV4, or \nFEV). Recently, increased
use of modern molecular methods based on next‐generation sequencing have enabled
the identification of distinct subgroups within this previously uncharacterized
group of Ewing‐like sarcomas based on the discovery of novel molecular driving events.
The focus of this review is to provide an update on the main subcategories of Ewing‐like
sarcomas discovered to date: \nCIC‐rearranged sarcomas, \nBCOR‐rearranged sarcomas,
sarcomas with a rearrangement between \nEWSR1 and a non‐ETS family gene, and the
substantial fraction of tumors which remain uncharacterized by molecular methods.
There is increasing evidence that these tumors represent stand‐alone entities with
unique characteristics rather than simply a subgroup of Ewing sarcoma; thus, the
question of the best therapeutic approach for these often aggressive sarcomas remains
of primary importance. Ultimately, large collaborative efforts will be necessary
to better determine the characteristics of this rare, heterogeneous family of tumors."
- Journal of Cellular Physiology, EarlyView.