Histology-driven chemotherapy of soft-tissue sarcoma

Soft-tissue sarcomas are rare diseases with >50 subtypes. Surgery is the most important treatment in localized disease, sometimes combined with radiotherapy. Chemotherapy is used as palliation in advanced disease, sometimes also with a potential to decrease tumour size and eradicate micro-metastases, making meaningful surgery possible. The role of chemotherapy as adjuvant treatment in localized disease is not finally settled. Doxorubicin and ifosfamide are the two drugs with the best established response rates in soft-tissue sarcoma, and a combination of these drugs has been a 'gold standard' for several years. However, there is an emerging knowledge of the biology and sensitivity to treatment for different histological subtypes. New drugs such as gemcitabine, taxanes and trabectedin have been explored in several studies, showing promising results. Even if most studies have encompassed many different subtypes and were limited in size, knowledge related to specific treatment for different subtypes is emerging. Examples are trabectedin in lyposarcoma and leiomyosarcoma, and taxanes in angiosarcoma.