Treatment of advanced soft tissue sarcoma by histological subtype: wish, prediction or reality?

Abstract

Soft tissue sarcomas (STS) are heterogenous cancers encompassing more than 100 histological and molecular subtypes. Their extreme rarity underscores the need for international collaboration to identify specific treatment protocols. Increasing knowledge of STS complexity as defined by molecular biology has led to the introduction of targeted therapies for several sarcoma subtypes, which is an encouraging start. In advanced STS, doxorubicin-based regimens are standard first-line chemotherapy. Options for second and later lines include ifosfamide, trabectedin, pazopanib, eribulin and gemcitabine-based regimens. Histological subtype has become a key factor when selecting best options to treat advanced sarcoma; however, the challenges of identifying optimal treatments for all STS histotypes are undeniably formidable. Fortunately, the sarcoma community shares the common goal of seeking greater knowledge about the characteristics of each subtype in order to improve diagnosis and outcomes. Progress made to date in this regard suggests that the vision to treat by subtype is achievable.