腹膜后脂肪肉瘤病因学研究进展  

Advances in the etiology of retroperitoneal liposarcoma

作　　者：袁志青 刘颖斌 Yuan Zhiqing;Liu Yingbin(Department of Biliary-Pancreatic Surgery,Renji Hospital,School of Medicine,Shanghai Jiao Tong University,Shanghai Cancer Research Institute,Shanghai 200127,China)

机构地区：[1]上海交通大学医学院附属仁济医院胆胰外科、上海市肿瘤研究所,上海200127

出　　处：《中华外科杂志》2024年第5期472-476,共5页Chinese Journal of Surgery

基　　金：2023仁济医院培育项目(RJTJ23-PY-6034)。

摘　　要：腹膜后脂肪肉瘤是最常见的腹膜后软组织肿瘤,起病隐匿,治疗困难,极易复发。不同亚型的腹膜后脂肪肉瘤在致病基因、生物学行为和预后方面差异明显。高分化和去分化脂肪肉瘤的基因组特征是12号染色体部分长臂片段的扩增。黏液样脂肪肉瘤的基因组特征为12号和16号染色体部分片段的相互易位形成融合基因。多形性及黏液多形性脂肪肉瘤的基因组改变复杂,存在多发的染色体结构异常。目前已发现多个与脂肪细胞分化或脂质代谢相关的信号转导通路可能与腹膜后脂肪肉瘤的发生和进展有关。腹膜后脂肪肉瘤起源于幼稚的前脂肪细胞还是去分化的成熟脂肪细胞还不明确,对其代谢特征的了解也不够深入。腹膜后脂肪肉瘤的一线药物治疗为以蒽环类药物为主的全身化疗,但患者获益不佳,亟须加强针对腹膜后脂肪肉瘤的基础研究以寻找有效的治疗靶点。Retroperitoneal liposarcoma is the most common retroperitoneal soft tissue tumor with insidious onset,difficulty in treatment,and easy recurrence.Different subtypes of retroperitoneal liposarcoma differ significantly in pathogenic mechanism,biological behavior,and prognosis.The characteristic molecular event of well-differentiated and dedifferentiated liposarcoma is the amplification of the long arm segment of chromosome 12.The genome of myxoid liposarcoma is characterized by translocations of chromosomes 12 and 16 to form fusion genes.The genomic changes of pleomorphic and myxoid pleomorphic liposarcoma are complex,with multiple chromosomal structural abnormalities.Several signaling pathways related to adipocyte differentiation or lipid metabolism have been found to be involved in the initiation and progression of retroperitoneal liposarcoma.It is unclear whether retroperitoneal liposarcoma originates from naive preadipocytes or dedifferentiated mature adipocytes,and its metabolic characteristics are also poorly understood.The first-line drug treatment for retroperitoneal liposarcoma is anthracycline-based chemotherapy,but patients receive little benefit.Therefore,it is urgent to strengthen the basic research on retroperitoneal liposarcoma to find effective therapeutic targets.

关 键 词：脂肪肉瘤 腹膜后间隙 基因组 病因学 

分 类 号：R735.4[医药卫生—肿瘤]