Beyond SMARCB1 Loss: Recent Insights into the Pathobiology of Epithelioid Sarcoma

Round 1

Reviewer 1 Report

This review summarizes the recent findings about ES pathobiology, including defects in chromatin remodeling and other signaling pathways and their role as therapeutic vulnerabilities.

The manuscript is well written and organized. The review will help the readers understand the molecular defects concur to its malignant progression.

Author Response

We thank the reviewer for his/her appreciation of our paper.

Reviewer 2 Report

In this well written and adequately illustrated review, the authors address the extremely rare but highly malignant Epithelioid sarcoma (ES) that displays ambiguous histopathologic features and is mainly characterized by the loss of SMARCB1/INI1 protein expression. The authors discuss the clinicopathological traits of ES including defects in chromatin remodeling and signaling pathways and elaborate on the putative exploitation of current ES knowledge into oncotherapeutic applications against this aggressive tumor type.

Minor comments:

LINE 105: Please denote what EWSR1 stands for

LINE 132: Do the authors mean the VA-ES-BJ cell line?

Author Response

We thank the reviewer for his/her appreciation of our paper. We have modified the text according to the reviewer's comments.  Specifically:

LINE 105: the definition of EWSR1 is now included (EWS RNA Binding Protein 1). 

LINE 132: we have specified that VA-ES-BJ refers to the VA-ES-BJ cell line