Max-Eder Research Group for Pediatric Sarcoma Biology, Institute of Pathology, Faculty of Medicine, LMU Munich, Munich, Germany.
Division of Translational Pediatric Sarcoma Research, German Cancer Research Center (DKFZ), Hopp Children's Cancer Center (KiTZ), German Cancer Consortium (DKTK), Heidelberg, Germany.
EMBO Mol Med. 2020 Nov 6;12(11):e11131. doi: 10.15252/emmm.201911131. Epub 2020 Oct 13.
Sarcomas are heterogeneous and clinically challenging soft tissue and bone cancers. Although constituting only 1% of all human malignancies, sarcomas represent the second most common type of solid tumors in children and adolescents and comprise an important group of secondary malignancies. More than 100 histological subtypes have been characterized to date, and many more are being discovered due to molecular profiling. Owing to their mostly aggressive biological behavior, relative rarity, and occurrence at virtually every anatomical site, many sarcoma subtypes are in particular difficult-to-treat categories. Current multimodal treatment concepts combine surgery, polychemotherapy (with/without local hyperthermia), irradiation, immunotherapy, and/or targeted therapeutics. Recent scientific advancements have enabled a more precise molecular characterization of sarcoma subtypes and revealed novel therapeutic targets and prognostic/predictive biomarkers. This review aims at providing a comprehensive overview of the latest advances in the molecular biology of sarcomas and their effects on clinical oncology; it is meant for a broad readership ranging from novices to experts in the field of sarcoma.
肉瘤是一种异质性的、具有临床挑战性的软组织和骨癌。尽管肉瘤仅占所有人类恶性肿瘤的 1%,但它们是儿童和青少年中第二常见的实体肿瘤类型,也是重要的继发性恶性肿瘤群体。迄今为止,已经确定了 100 多种组织学亚型,并且由于分子分析,还发现了更多的亚型。由于其大多数具有侵袭性的生物学行为、相对罕见性以及几乎发生在每个解剖部位,许多肉瘤亚型都属于特别难以治疗的类别。目前的多模式治疗方案结合了手术、多化疗(伴/不伴局部热疗)、放疗、免疫疗法和/或靶向治疗。最近的科学进展使人们能够更精确地对肉瘤亚型进行分子特征分析,并揭示了新的治疗靶点和预后/预测生物标志物。本文旨在全面概述肉瘤的分子生物学的最新进展及其对临床肿瘤学的影响;它适合于从肉瘤领域的新手到专家的广泛读者。