New insights in the immuno- and molecular biology of soft tissue and bone tumors with therapeutic implications

Soft tissue tumor and tumors of the bone are rare neoplasm’s. The sarcoma account for less than 1% while bone sarcoma only for 0.2% of all malignant tumors. Nowadays, surgery remains the mainstay of treating localized disease. Combination with adjuvant radiation for larger and unresectable tumors is the therapy of choice. Chemotherapy is used in specific subtypes, such as rhabdomyosarcoma, osteosarcoma and Ewing´s sarcoma; however, effective treatment of advanced sarcoma remains a challenge. About 10% of the tumors have detectable metastases by the time of diagnosis of the primary tumor. Furthermore, depending upon stage and subtype, 25-50% of patients develop recurrent and/or metastatic disease. The etiology of most soft tissue and bone tumors is unknown. It has been suggested that genetic and environmental factors, irradiation, viral infections and immunodeficiency are associated with the development of those tumors. Advances in understanding the genetic nature en biology of cancer have led to the development of new treatment options for sarcoma. Tyrosine kinase inhibitors (TKI´s) have so far been used to suppress the tyrosine kinase effect on tumor progression and this way to control the evolution of the disease. The TKI´s have been used in treating soft tissue sarcomas, with main representative GIST´s, but their use remains restricted due to the lack of data on mesenchymal tumors. Moreover, the immune system is crucial in cancer control and progression, and appropriate modulation of the immune system may provide an effective therapeutic option for sarcoma. Thus far, no effective immunological therapy for sarcoma has been identified. Our goal with this study is to investigate the nature and biology of soft tissue and bone tumors and to adjust the results of the study on the clinical setting, in order to have more effective control and/or complete remission of this therapeutic challenging neoplasms.

Publication year:2021

Keywords:Doctoral thesis

Accessibility:Open