Soft tissue sarcoma

Soft tissue sarcomas belong to the most challenging diseases in oncology amounting to only 1% of all cancers. Soft tissue sarcomas are rare malignant disorders that comprise an extremely heterogenous spectrum of tumors. With the improvement of surgical techniques and radiation therapy the majority of patients with localize disease can be cured. However, for patients with advanced or metastasized disease chemotherapeutic treatments have not greatly changed the poor outcome of the disease.The introduction of combined chemoradiotherapy as well as the isolated limb-perfusion has improved the limb-salvage rate in locally advanced disease but the impact of systemic chemotherapy on overall survival remains still 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. 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. 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.