Soft tissue sarcomas (STS) are a heterogeneous group of rare malignancies frequently studied and treated as if they were one and the same disease. Evidence is emerging that distinct histopathological differences between the subtypes can significantly impact on optimal management of patients with STS. For the majority of patients with localized disease, surgery is the treatment of choice, sometimes combined with radiotherapy. For patients with advanced/refractory disease, there are a number of options. The first option is to consider cytotoxic chemotherapy with doxorubicin ± ifosfamide to reduce tumor size and make the tumor more amenable to surgery. If this is not possible, treatment should be aimed at reducing symptoms, improving patients' wellbeing and prolonging life. In this regard, understanding of the different biologies and sensitivities of the various histological subtypes of STS continues to expand, and an increasing number of targeted therapies are becoming available. Examples of more specific treatment options include taxanes in angiosarcoma, and trabectedin in leiomyosarcoma, liposarcoma and undifferentiated pleomorphic sarcoma. Although much remains to be learned about these rare malignancies, it is anticipated that small steps taken in recent years will lead to bigger leaps forward in future.
Keywords: chemotherapy; doxorubicin; histology; ifosfamide; leiomyosarcoma; soft tissue sarcoma; trabectedin.