Retroperitoneal liposarcomas are rare tumours that carry a poorer prognosis than their extremity counterparts. Within their subtypes - well differentiated (WDL), dedifferentiated (DDL), myxoid (MLS) and pleomorphic (PLS) - they exhibit a diverse genomic landscape. With recent advances in next generation sequencing, the number of studies exploring this have greatly increased. The recent literature has deepened our understanding of the hallmark MDM2/CDK4 amplification in WDL/DDL and addressed concerns about toxicity and resistance when targeting this. The FUS-DDIT3 fusion gene remains the primary focus of interest in MLS with additional potential targets described. Whole genome sequencing has driven identification of novel genes and pathways implicated in WDL/DDL outside of the classic 12q13-15 amplicon. Due to their rarity; anatomical location and histologic subtype are infrequently mentioned when reporting the results of these studies. Reports can include non-adipogenic or extremity tumours, making it difficult to draw specific retroperitoneal conclusions. This narrative review aims to provide a summary of retroperitoneal liposarcoma genomics and the implications for therapeutic targeting.

中文翻译：

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腹膜后脂肪肉瘤基因组学综述。

腹膜后脂肪肉瘤是罕见的肿瘤，其预后较四肢为差。在它们的亚型中-高分化（WDL），去分化（DDL），类固醇（MLS）和多形性（PLS）-它们展现出多样化的基因组格局。随着下一代测序技术的最新发展，探索该技术的研究数量已大大增加。最近的文献已经加深了我们对WDL / DDL中标志性MDM2 / CDK4扩增的理解，并解决了针对此的毒性和耐药性问题。FUS-DDIT3融合基因仍然是MLS的主要关注焦点，并描述了其他潜在靶标。全基因组测序已驱动鉴定出经典12q13-15扩增子之外的WDL / DDL中涉及的新基因和途径。由于它们的稀有性；报告这些研究结果时，很少提及解剖位置和组织学亚型。报告可能包含非脂肪性或四肢肿瘤，因此很难得出具体的腹膜后结论。这篇叙述性综述旨在提供腹膜后脂肪肉瘤基因组学的概述及其对治疗靶向的意义。