Standards and novel therapeutic options in the treatment of patients with soft tissue sarcoma

Soft tissue sarcomas are a heterogeneous group of tumours arising predominantly from the embryonic mesoderm. They account for less than 1% of all adult malignancies. The prognosis of patients with advanced metastatic soft tissue sarcoma remains poor with a disease-free survival at 5 years less than 10%. Despite improvements in local tumour control due to surgery and radiotherapy, distant metastasis and death remain a significant problem in 50% of patients. Complete resection remains the major factor in providing cure with limited benefits in local tumour control by radiotherapy and only minimal benefit of systemic therapy for metastatic disease. Only few chemotherapeutic agents have been identified to be active with reported response rates for doxorubicin and ifosfamide around 20%. New strategies are urgently needed to improve patients' outcome. Progress in the molecular characteristics, the understanding of biology and pathogenesis of these tumours has been made and should in the near future translate into molecularly based therapies. We describe current treatment strategies and existing standards. Moreover, we give an overview on the emerging compounds for patients with soft tissue sarcoma including recent developments of targeted therapy focusing on antiangiogenic and immunomodulatory drugs, Bcl-2 antisense therapy, raf kinase and mTOR inhibition, minor groove binders, and other agents being developed.