Defining the Extracellular Matrix of Rhabdomyosarcoma

Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma of childhood with a propensity to metastasize. Current treatment for patients RMS includes conventional systemic chemotherapy, radiation therapy, and surgical resection; nevertheless, little no improvement in long term survival has been achieved decades—underlining need target discovery new therapeutic approaches targeting tumor cells or microenvironment. To evaluate cross-species extracellular matrix production, we have used murine models which feature knowledge myogenic cell-of-origin. With focus on RMS/undifferentiated pleomorphic (UPS) continuum, constructed tissue microarrays 48 four human sarcomas analyze expression seven different collagens, fibrillins, collagen-modifying proteins, cross-correlation RNA deep sequencing. We uncovered that produces increased type XVIII collagen alpha 1 (COL18A1), clinically associated decreased long-term survival. also identified significantly COL4A1, FBN2, PLOD1, PLOD2 relative normal skeletal muscle. These results complement recent studies investigating whether soft utilize enzymes alter structural integrity surrounding host matrix/collagen quaternary structure resulting improved ability improve invade regionally metastasize, possible.