Soft tissue sarcoma (STS) is a rare neoplasm, often neglected in funding programs, but is relatively frequent in children and the young, and has, therefore, a relevant public health and societal impact.
Diagnosis is often delayed, altering treatment, increasing disability, and worsening prognosis. To date, the etiology of STS is largely unknown, because epidemiological studies are limited and with low statistical power.
This unique interdisciplinary project is based on a large investigation of the genomic, epidemiologic, toxicological, and clinical aspects of STS. A multicentric case-control study including interviewer-based data and biological samples has been conducted since 2011, and further data collection will be continued for the proposed project. The large amount of data and biological samples will allow to (1) identify and quantify various modifiable and not modifiable risk factors, including defects in mitotic and telomere biology, for STS; (2) assess the association of dioxin, furans, and selected dioxin-like compounds (polychlorobiphenyls, CBs), and oncogenic viruses with STS; (3) characterize the clinical history of STS before correct diagnosis to identify common drawbacks in the diagnostic pathway. The project will be in the condition of integrating biomarkers and epidemiological information; its results will have a comprehensive impact on our knowledge of STS determinants and, consequently, prevention and management.