GINECO/TMRO and NETSARC Network, Center Léon Bérard & Université Claude Bernard, Lyon, France.
Department of Oncology, Helios Klinikum Berlin-Buch, Schwanebecker Chaussee 50, 13125, Berlin, Germany.
Future Oncol. 2018 May;14(10s):25-49. doi: 10.2217/fon-2018-0076.
Management of soft tissue sarcoma is increasingly subtype-dependent. Surgery is recommended for uterine leiomyosarcoma, with trabectedin being the preferred option for advanced disease when the treatment goal is long-term tumor stabilization. Liposarcoma subgroups are characterized by distinctive morphologies and genetics, different patterns of disease progression and clinical behavior, and variable responses to treatment. Genetic analysis of sarcomas has provided insights into pathogenesis with potential for developing new molecular targets. At the cytogenetic level, soft tissue sarcomas are categorized into specific, balanced translocations and those due to massive chromosomal rearrangements. For subtypes such as undifferentiated sarcomas, angiosarcomas, alveolar soft part sarcomas and clear cell sarcomas, evidence is especially limited, although it is known that these tumors display markedly different sensitivities to chemotherapeutic and targeted agents.
软组织肉瘤的治疗越来越依赖于亚型。对于子宫平滑肌肉瘤，建议手术治疗，当治疗目标是长期肿瘤稳定时，曲贝替定为晚期疾病的首选药物。脂肪肉瘤亚组的特点是形态和遗传学上的独特性、不同的疾病进展和临床行为模式，以及对治疗的不同反应。肉瘤的基因分析为发病机制提供了新的见解，具有开发新的分子靶点的潜力。在细胞遗传学水平上，软组织肉瘤分为特定的、平衡的易位和由于大量染色体重排引起的易位。对于未分化肉瘤、血管肉瘤、腺泡状软组织肉瘤和透明细胞肉瘤等亚型，证据尤其有限，尽管已知这些肿瘤对化疗药物和靶向药物的敏感性明显不同。
