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VOL. 5, N. 2, 2017 Background Soft tissue sarcomas (STS) are a rare group of tumors of mesenchymal origin comprising about 1% of all malignancies in the adulthood. According to the updated WHO classification (2013), STS represent a highly heterogeneous tumor entity of more than 50 subtypes based on their histological, molecular and certainly clinical characteristics [1]. Conventional chemotherapy with doxorubicin and/or ifosfamide still represents the backbone of systemic treatment in the locally advanced and metastatic setting sequentially or in combination [2]. Since the early 80’s many trials have been published investigating the addition of new drugs to doxorubicin in order to improve overall survival (OS). However, no statistically significant benefit could be proven regarding this matter. Even in the European Organisation for Research and Treatment of Cancer (EORTC) Soft Tissue and Bone Sarcoma Group (STBSG) 62012 trial that included 455 patients and compared single agent doxorubicin with a combination regimen with ifosfamide in a randomized fashion, the primary endpoint of improvement in OS was not met. Whereas the response rate was higher (27% vs 14%) and the progression-free survival (PFS) could be prolonged in the combination arm (7.4 vs 4.6 months), the doxorubicin/ifosfamide combination did not lead to a statistically significant improvement in OS (14.3 vs 12.8 months) [3]. For the first time after 40 years of standard first-line anthracycline therapy, the approval of olaratumab, a platelet-derived growth factor receptor (PDGFR) inhibitor, in combination with doxorubicin, has revolutionized the first-line treatment of advanced STS [4]. In a randomized phase II trial, olaratumab combined with doxorubicin was able to prolong OS for almost one year when compared to doxorubicin alone. With the successful launching of trabectedin, pazopanib and eribulin for specific subtypes of STS, the treatment landscape in the further-line setting has been broadened and promising systemic treatment options can be offered to patients [5-8]. Furthermore, taxanes have shown a beneficial response in angiosarcomas, and leiomyosarcomas seems to be highly sensitive to gemcitabine alone or combined with docetaxel [9-10]. With the rapidly evolving knowledge on sarcoma pathogenesis and increasing clinical trial data on treatment of sarcoma subtypes, histology-driven chemotherapy is already well-established in the present and represents the future. A simplified treatment algorithm illustrating potential systemic treatment options for locally advanced and metastatic STS is depicted in Figure 1. Abstract Soft tissue sarcomas are rare tumors of mesenchymal origin comprising about 1% of all adult malignant diseases. Systemic therapy for locally advanced and metastatic disease was restricted for decades to a few effective and approved agents, such as doxorubicin or ifosfamide. However, numerous clinical trials and new drug developments such as trabectedin, pazopanib, olaratumab or eribulin have recently enriched the therapeutic armamentarium in the treatment of patients with advanced soft tissue sarcomas and will be presented in the following review.

Metastatic soft tissue sarcomas: update on new agents and clinical studies