Soft tissue sarcomas are rare tumors that represent a major challenge due to 
varying clinical presentations and often interdisciplinary treatment concepts. 
Gold standard for the treatment of localized resectable soft tissue sarcomas is 
complete surgical removal. In metastatic soft tissue sarcoma, systemic therapy 
is the treatment of choice. The most active drugs are anthracyclines and 
ifosfamide. Combination chemotherapy has improved both response rate and 
progression-free survival at the cost of increased toxicity. Imatinib at a dose 
of 400 mg/day is the gold standard for patients with advanced or metastatic 
gastrointestinal stromal tumors (GIST). In patients with a mutation in KIT 
exon 9, 800 mg/day is the recommended dose. In imatinib refractory or intolerant 
patients, sunitinib is recommended. Regorafenib has been approved for third-line 
therapy.