Sarcoma articles within Nature Reviews Clinical Oncology

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  • Review Article |

    Gastrointestinal stromal tumour (GIST) is the most common form of sarcoma and has become a paradigm of precision medicine owing to the fact that almost all patients harbour one of several known molecule drivers, most of which can be targeted therapeutically. Nevertheless, novel therapeutic strategies are required to overcome the intrinsic resistance of certain subtypes of GIST to existing treatments as well as the acquired resistance that eventually arises in initially sensitive subtypes. This Review describes the biology of GIST, the evolution of the current treatments for this cancer, and the emerging therapeutic agents and approaches that might overcome the remaining clinical challenges.

    • Lillian R. Klug
    • , Homma M. Khosroyani
    •  & Michael C. Heinrich

  • Review Article |

    Despite being the most common primary bone cancer in children and young adults, osteosarcoma is a rare cancer, a fact that has complicated efforts to improve patient outcomes. Moreover, the molecular biology of disease is highly heterogeneous and most of the recurrent genetic alterations occur in tumour-suppressor genes that are challenging therapeutic targets. Herein, Gill and Gorlick discuss the new biological discoveries, technologies, and therapeutic agents and approaches that, through collaborative efforts, are poised to generate advances in the treatment of osteosarcoma after more than four decades of stagnation.

    • Jonathan Gill
    •  & Richard Gorlick

  • Review Article |

    Patients with sarcomas have historically been treated with surgery and/or chemotherapy, although the outcomes achieved with these approaches, especially in advanced-stage disease, are often disappointing. In this Review, the authors describe the opportunities created by selective use of targeted therapies on the basis of the biological characteristics of individual tumours.

    • Armelle Dufresne
    • , Mehdi Brahmi
    •  & Jean-Yves Blay

  • News & Views |

    Patients with metastatic soft-tissue sarcoma can benefit from systemic therapy, but the best drug combinations for the different disease subtypes remain to be established. Recently, great emphasis has been placed on histology-based chemotherapy regimens. Herein, we discuss the results of a recently published study demonstrating that some of these regimens are not superior to standard-of-care chemotherapy in the neoadjuvant setting.

    • Robin L. Jones
    •  & Khin Thway

  • Year in Review |

    In 2016, novel findings on the role of predisposing gene variants in sarcoma oncogenesis were published, as well as studies addressing novel molecular classifications and results from randomized controlled trials highlighting successful new treatments. Herein, we discuss these meaningful advances.

    • Jean-Yves Blay
    •  & Isabelle Ray-Coquard

  • News & Views |

    In a recent study, the addition of olaratumab to doxorubicin chemotherapy for patients with soft-tissue sarcoma resulted in prolongation of progression-free survival by only 2.5 months, but an overall survival benefit of 11.8 months; the large disparity between these outcomes raises important questions. We discuss these results in relation to those of other trials, and the implications for sarcoma therapy.

    • Ian Judson
    •  & Winette T. van der Graaf

  • Review Article |

    Soft-tissue sarcomas (STS) are a rare and heterogeneous group of tumours, with a wide range of differing behaviours and underlying molecular pathologies. Recent advances in molecular pathogenesis, novel targeted therapies, changes in clinical trial design and increased international collaboration have led to the development of histology-driven therapy. The authors of this Review describe the current gold standard treatment for specific STS subtypes and outline the future promising therapies in the pipeline.

    • Mark Linch
    • , Aisha B. Miah
    •  & Charlotte Benson

  • News & Views |

    Nomograms have been constructed to provide patient-specific predictions for overall survival and disease-free survival after resection for patients with primary localized retroperitoneal soft tissue sarcoma, by combining data registered across three institutional databases. However, whether overall survival, or disease-free survival, is a more useful predictor is disputable.

    • Murray F. Brennan

  • News & Views |

    The PALETTE randomized phase III trial has reported a statistically significant benefit for the use of pazopanib compared to placebo in terms of progression-free survival for the treatment of soft-tissue sarcoma. However, no benefit in terms of overall survival was observed, the reasons for which might lie in the study design.

    • Isabelle Ray-Coquard
    •  & David Thomas

  • Opinion |

    Many patients with soft-tissue sarcomas are treated with doxorubicin-based adjuvant chemotherapy, but whether this therapy translates into a survival benefit is controversial. The authors of this Perspectives article critically analyze available clinical data and discuss the implications of these data on current and future treatment efforts.

    • Igor Matushansky
    •  & Robert N. Taub

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