Abstract
Soft tissue sarcomas are rare cancers of mesenchymal origin or differentiation comprising over 70 different histological subtypes. Due to their mesenchymal differentiation, sarcomas are thought to produce and deposit large quantities of extracellular matrix (ECM) components. Interactions between ECM ligands and their corresponding adhesion receptors such as the integrins and the discoidin domain receptors play key roles in driving many fundamental oncogenic processes including uncontrolled proliferation, cellular invasion and altered metabolism. In this review, we focus on emerging studies that describe the key ECM components commonly found in soft tissue sarcomas and discuss preclinical and clinical evidence outlining the important role that these proteins and their cognate adhesion receptors play in sarcomagenesis. We conclude by providing a perspective on the need for more comprehensive in-depth analyses of both the ECM and adhesion receptor biology in multiple histological subtypes in order to identify new drug targets and prognostic biomarkers for this group of rare diseases of unmet need.
Highlights
Soft tissue sarcomas (STS) are a group of rare cancers accounting for 1% of adult cancers and 15% of cancers in children and adolescents (Mastrangelo et al, 2012; Howlader et al, 2020)
This study suggests that the interaction of NG2+ sarcoma cells with stroma rich in collagen VI might be driving STS cell migration which may explain the observed association of NG2 expression levels with a higher risk of postsurgical metastasis
Much more work is required to understand the biological significance of extracellular matrix (ECM)-tumour cell interactions in this group of rare diseases
Summary
Soft tissue sarcomas (STS) are a group of rare cancers accounting for 1% of adult cancers and 15% of cancers in children and adolescents (Mastrangelo et al, 2012; Howlader et al, 2020). In the tumours of peripheral nerve origin (6 neurofibroma, 6 schwannoma and 2 malignant schwannomas), the staining was weak and irregular It remains to be determined if the differential collagen III staining observed is due to distinct cellular origin (smooth muscle versus nerve) of these subtypes and if collagen III has a role in the growth and progression of the different soft tissue tumours. Another class of collagens is the network forming collagens. Collagen IV detected in MPNST and epithelioid areas of SS
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