The standard treatment for patients with localized STS is surgery. The main goal of surgery is the en-bloc resection of the tumor mass with adequate margins (1-2 cm) using conservative surgical procedures.

Radiation therapy and chemotherapy are complementary treatments and they can be used prior or after surgery, depending on the size of the tumor, on the chances of achieving a radical resection using conservative procedures, and considering also the adequacy of margins and the histological grade of the tumor. Radiation therapy provides a local control of the disease in 80-85% of cases. It is generally used after surgery since it can interfere with physiological healing process, for that reason it is used before surgery only in patients with borderline resectable tumors in order to increase the chances of complete resections. Similarly, chemotherapy prior surgery is recommended in case of bulky masses in order to increase the chances of a conservative surgery with the hope to avoid demolitive surgery, whenever possible.

The role of adjuvant chemotherapy is still controversial. In general, prior to start a chemotherapy clinicians evaluate multiple risk factors (deep location, size, histological grade) and discuss about it with the patient. The treatment for metastatic disease is chemotherapy when metastasis are visceral, hepatic or located in the bones. For lung metastasis without other locations of disease a local therapy as surgical resection or stereotactic radiation therapy can be evaluated.

The most used drugs are anthracycline, ifosfamide, gemcitabine, taxotere, or trabectedine – a new generation drug currently approved as second line therapy or as first line therapy for patients that cannot receive anthracycline as first line therapy. Also, targeted therapies like sunitinib, sorafenib and R1507 can be a chance for selected histotypes of STS.

Chemosensitivity depends on the histology of the tumor, which means that the histological features of the tumor lead more and more the choice of the treatment.