Well-differentiated liposarcomas (WDLPS) and dedifferentiated liposarcomas (DDLPS) account for 60 % of all liposarcomas, reflecting the heterogeneity of this type of sarcoma. Genetically, both types of liposarcomas are characterized by the amplification of and genes, which indicates an important molecular event with diagnostic and therapeutic relevance. In both localized WDLPS and DDLPS of the retroperitoneum and the extremities, between 25 % and 30 % of patients have local or distant recurrence, even when perioperatively treated, with clear margins present. The systemic treatment of WDLPS and DDLPS remains a challenge, with anthracyclines as the gold standard for first-line treatment. Several regimens have been tested with modest results regarding their efficacy. Herein we discuss the systemic treatment options for WDLPS and DDLPS and review their reported clinical efficacy results.

中文翻译：

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高分化脂肪肉瘤和去分化脂肪肉瘤：两种同胞肿瘤的全身治疗选择

高分化脂肪肉瘤（WDLPS）和去分化脂肪肉瘤（DDLPS）占所有脂肪肉瘤的60%，反映了该类型肉瘤的异质性。从遗传学上来说，两种类型的脂肪肉瘤都以 和 基因的扩增为特征，这表明具有诊断和治疗相关性的重要分子事件。在腹膜后和四肢的局部 WDLPS 和 DDLPS 中，即使在围手术期治疗且边缘清晰的情况下，25% 至 30% 的患者也会出现局部或远处复发。 WDLPS 和 DDLPS 的全身治疗仍然是一个挑战，蒽环类药物是一线治疗的金标准。已经对几种治疗方案进行了测试，其疗效结果有限。在此，我们讨论 WDLPS 和 DDLPS 的系统治疗方案，并回顾其报告的临床疗效结果。