Division of Molecular Pathology, The Institute of Cancer Research, London, United Kingdom.
Division of Molecular Pathology, The Institute of Cancer Research, London, United Kingdom; The Royal Marsden NHS Foundation Trust, London, United Kingdom.
Crit Rev Oncol Hematol. 2023 Apr;184:103955. doi: 10.1016/j.critrevonc.2023.103955. Epub 2023 Mar 7.
Leiomyosarcoma (LMS) is a soft tissue sarcoma of smooth muscle origin that can arise in multiple anatomical sites and is broadly classified as extra-uterine LMS or uterine LMS. There is substantial interpatient heterogeneity within this histological subtype, and despite multi-modal therapy, clinical management remains challenging with poor patient prognosis and few new therapies available. Here we discuss the current treatment landscape of LMS in both the localised and advanced disease setting. We further describe the latest advances in our evolving understanding of the genetics and biology of this group of heterogeneous diseases and summarise the key studies delineating the mechanisms of acquired and intrinsic chemotherapy resistance in this histological subtype. We conclude by providing a perspective on how novel targeted agents such as PARP inhibitors may usher in a new paradigm of biomarker-driven therapies that will ultimately impact the outcomes of patients with LMS.
平滑肌肉瘤（LMS）是一种源自平滑肌的软组织肉瘤，可以发生在多个解剖部位，通常分为子宫外 LMS 或子宫 LMS。这种组织学亚型的患者间存在很大的异质性，尽管采用了多模式治疗，但临床管理仍然具有挑战性，患者预后较差，可用的新疗法很少。本文讨论了局限性和晚期疾病中 LMS 的当前治疗现状。进一步描述了我们对这群异质性疾病的遗传学和生物学认识的最新进展，并总结了阐明该组织学亚型获得性和内在化疗耐药机制的关键研究。最后，我们展望了新型靶向药物，如 PARP 抑制剂如何开创生物标志物驱动治疗的新模式，从而最终影响 LMS 患者的治疗结果。
