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

Abstract

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