Rhabdomyosarcoma--working out the pathways.

摘要: Rhabdomyosarcomas constitute a collection of childhood malignancies thought to arise as consequence regulatory disruption skeletal muscle progenitor cell growth and differentiation. Our understanding the pathogenesis this neoplasm has recently benefited from study normal malignant myogenic cells in vitro, facilitating identification diagnostic cytogenetic markers elucidation mechanisms by which myogenesis is regulated. It now appreciated that delicate balance between proliferation differentiation, mutually exclusive yet intimately associated processes, normally controlled large part through action multitude factors, whose signals are interpreted members MyoD family helix – loop – helix proteins, key cycle factors. The latter have proven be frequent targets mutational events subvert promote development rhabdomyosarcoma. Although significant progress been made treatment rhabdomyosarcoma, patients presenting with metastatic disease or certain high risk features still faced dismal prognosis. Only genetically engineered mouse models becoming available provide fresh insights into molecular/genetic pathways rhabdomyosarcomas progress, suggest novel avenues therapeutic opportunity.