Nonrhabdomyosarcoma Soft Tissue Sarcoma in Children: Developing New Treatments Based on a Better Understanding of Disease Biology

摘要: Nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) are a large and very ­heterogeneous group of cancers in children. Although approximately 550 NRSTS diagnoses the US each year represent only 4% cases childhood cancer, comprise nearly 60% all this age [reviewed Spunt et al. (2006)]. The incidence is bimodally distributed, with relatively high first life second peak during later adolescence. While typically sporadic disease, some types associated cancer susceptibility syndromes, such as Li–Fraumeni familial syndrome (associated heritable p53 mutations) neurofibromatosis type I mutations NF1 tumor suppressor genes) (Li 1988; Malkin 1990; Sorensen 1986); certain environmental exposures (therapeutic ionizing radiation HHV8, HIV, or EBV virus infection); chemical carcinogens (Spunt 2006).