Cancers, Vol. 16, Pages 1355: Functional Classification of Fusion Proteins in Sarcoma

Cancers, Vol. 16, Pages 1355: Functional Classification of Fusion Proteins in Sarcoma Cancers doi: 10.3390/cancers16071355 Authors: Marco Wachtel Didier Surdez Thomas G. P. Grünewald Beat W. Schäfer Sarcomas comprise a heterogeneous group of malignant tumors of mesenchymal origin. More than 80 entities are associated with different mesenchymal lineages. Sarcomas with fibroblastic, muscle, bone, vascular, adipocytic, and other characteristics are distinguished. Nearly half of all entities contain specific chromosomal translocations that give rise to fusion proteins. These are mostly pathognomonic, and their detection by various molecular techniques supports histopathologic classification. Moreover, the fusion proteins act as oncogenic drivers, and their blockade represents a promising therapeutic approach. This review summarizes the current knowledge on fusion proteins in sarcoma. We categorize the different fusion proteins into functional classes, including kinases, epigenetic regulators, and transcription factors, and describe their mechanisms of action. Interestingly, while fusion proteins acting as transcription factors are found in all mesenchymal lineages, the others have a more restricted pattern. Most kinase-driven sarcomas belong to the fibroblastic/myofibroblastic lineage. Fusion proteins with an epigenetic function are mainly associated with sarcomas of unclear differentiation, suggesting that epigenetic dysregulation leads to a major change in cell ...
Source: Cancers - Category: Cancer & Oncology Authors: Tags: Review Source Type: research