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[腹膜后脂肪肉瘤病因学的进展]

[Advances in the etiology of retroperitoneal liposarcoma].

作者信息

Yuan Z Q, Liu Y B

机构信息

Department of Biliary-Pancreatic Surgery, Renji Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai Cancer Research Institute, Shanghai 200127, China.

出版信息

Zhonghua Wai Ke Za Zhi. 2024 May 1;62(5):472-476. doi: 10.3760/cma.j.cn112139-20231018-00179.

Abstract

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.

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,且易复发。腹膜后脂肪肉瘤的不同亚型在致病机制、生物学行为和预后方面存在显著差异。高分化和去分化脂肪肉瘤的特征性分子事件是12号染色体长臂片段的扩增。黏液样脂肪肉瘤的基因组特征是12号和16号染色体易位形成融合基因。多形性和黏液样多形性脂肪肉瘤的基因组变化复杂,存在多种染色体结构异常。已发现几种与脂肪细胞分化或脂质代谢相关的信号通路参与腹膜后脂肪肉瘤的发生和发展。目前尚不清楚腹膜后脂肪肉瘤是起源于幼稚前脂肪细胞还是去分化的成熟脂肪细胞,其代谢特征也知之甚少。腹膜后脂肪肉瘤的一线药物治疗是以蒽环类为基础的化疗,但患者获益甚微。因此,迫切需要加强对腹膜后脂肪肉瘤的基础研究,以寻找有效的治疗靶点。

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