# Unveiling the myxofibrosarcoma tumor microenvironment: implications for immunotherapy

## TL;DR

This paper reviews the immune landscape of myxofibrosarcoma to identify potential biomarkers and new immunotherapy strategies for this aggressive cancer.

## Contribution

The paper provides a comprehensive review of immune-related biomarkers and ongoing clinical trials in myxofibrosarcoma.

## Key findings

- MFS shows variable expression of immune checkpoint molecules like PD-1 and PD-L1.
- Tumor-infiltrating lymphocytes and alterations in antigen presentation pathways are observed in MFS.
- Angiogenic signatures suggest a role for vascular remodeling in the tumor microenvironment.

## Abstract

Myxofibrosarcoma (MFS) is a rare and aggressive soft tissue sarcoma characterized by high genomic instability, resulting in high local recurrence rates and limited effective therapeutic options in advanced stages. Recent progress in cancer immunology research has encouraged investigation into the Tumor Microenvironment (TME) of sarcomas, including MFS, to identify immune-related biomarkers of prognostic and therapeutic relevance. Although data remain limited in MFS, existing evidence suggests a heterogeneous immune landscape, including: i) variable expression of immune checkpoint molecules such as Programmed Cell Death Protein 1 (PD-1) and Programmed Death-Ligand 1 (PD-L1), ii) presence of tumor-infiltrating lymphocytes, iii) alterations in antigen presentation pathways, and iv) a pronounced angiogenic signature. These findings underscore the potential role of immune biomarkers for patients’ clinical stratification and the consequent possibility of developing new immunotherapeutic strategies. This review will focus on the cellular and molecular architecture of immune infiltration, vascular remodeling, and lymphoid neogenesis, assessing their prognostic and predictive value as potential biomarkers. Finally, we will present ongoing clinical trials aimed at modulating the immune-vascular niche to inform innovative therapeutic strategies for this challenging sarcoma subtype.