The Royal Marsden Hospital, Sarcoma Unit, Fulham Road, SW3 6JJ, London, UK.
Expert Rev Anticancer Ther. 2014 Mar;14(3):297-306. doi: 10.1586/14737140.2014.869173. Epub 2014 Jan 8.
Liposarcoma (LS) represents one of the most common soft tissue sarcomas. There are three major subtypes, namely, well/dedifferentiated, myxoid/round cell and pleomorphic LS. In general, LS is known to be a relatively chemo-resistant sarcoma subtype with the exception of the myxoid variant. Conventional chemotherapy with doxorubicin and ifosfamide represents the mainstay of systemic treatment in the first line. Other active cytotoxic agents include gemcitabine and docetaxel and the marine-derived compounds trabectedin. Recent progress in molecular diagnostics of each single LS subtype has improved the knowledge of the molecular characteristics and has led to two recent treatment targets: the amplification of mouse double minute 2 homolog and cyclin-dependent kinase-4 in well- and dedifferentiated LS. Thus far, only early-phase trials are reported and no new drugs have been introduced in daily clinical practice. The focus of this review is on current systemic treatment options, including novel strategies.
脂肪肉瘤(LS)是最常见的软组织肉瘤之一。它有三个主要亚型,即高分化/去分化型、黏液样/圆形细胞型和多形性 LS。一般来说,LS 被认为是一种相对化疗耐药的肉瘤亚型,黏液样变异型除外。多柔比星和异环磷酰胺的常规化疗是一线治疗的主要方法。其他有效的细胞毒性药物包括吉西他滨和多西他赛以及海洋来源的化合物 trabectedin。每种 LS 亚型的分子诊断的最新进展提高了对分子特征的认识,并导致了最近的两个治疗靶点:高分化/去分化 LS 中鼠双微体 2 同源物和细胞周期蛋白依赖性激酶 4 的扩增。到目前为止,仅报道了早期试验,尚无新药在日常临床实践中应用。这篇综述的重点是当前的全身治疗选择,包括新的策略。