Locally advanced and metastatic sarcoma (adult type) including gastrointestinal stromal tumors

STS belong to the most challenging diseases in oncology that demand all resources of modern clinical oncology. With the improvement of surgical techniques and radiation therapy the majority of patients with localized disease can be cured. However, for patients with locally advanced or metastatic disease chemotherapeutic treatments have not greatly changed the poor outcome of the disease. The introduction of combined chemoradiotherapy as well as isolated limb-perfusion has improved the limb-salvage rate in locally advanced disease but the impact of systemic chemotherapy on overall survival remains a subject of dispute. For patients with metastatic sarcoma long-term survival can only be achieved in a small number of patients with mostly resectable disease. The list of effective drugs for palliative treatment in general still remains short and the duration of remissions usually does not exceed several months. The lack of alternative chemotherapeutic drugs imposes a considerable challenge in daily clinical practice with many young patients exhibiting a good performance status but progressive disease after standard treatment. A variety of new drugs or drug combinations seem to exhibit considerable activity in certain histological sarcoma subtypes, which may soon broaden the armamentarium of drugs for a subset of patients. However, with the vastly improved understanding of the biology and pathology of soft tissue sarcoma an era of opportunities seems to have begun and the recent success in the treatment of gastrointestinal stromal tumors impressively shows how fast a gain in the understanding of oncogenic mechanisms may translate into a highly efficient, clinically useful treatment.