脂肪肉瘤的临床病理和基因改变的研究进展  

Research progress on clinicopathological and genetic alterations of liposarcoma

作　　者：李赛阳 包俊杰(综述)[1] 曲国蕃(审校)[1] LI Saiyang;BAO Junjie;QU Guofan(Harbin Medical University Cancer Hospital,Harbin 150081,China)

机构地区：[1]哈尔滨医科大学附属肿瘤医院,哈尔滨150081

出　　处：《实用肿瘤学杂志》2023年第1期83-86,共4页Practical Oncology Journal

摘　　要：脂肪肉瘤是一种起源于原始间叶细胞的恶性肿瘤,具有显著的组织多样性,由分化和异型程度不同的细胞组成。脂肪肉瘤主要分为四个亚型:以MDM2-HMGA2为关键基因的非典型脂肪源性肿瘤(Atypical lipogenic tumors,ALT)/高分化脂肪肉瘤(Well-differentiated liposarcoma,WDLPS)和去分化脂肪肉瘤(Dedifferentiated liposarcoma,DDLPS),以FUS-CHOP基因融合为主要发生机制的黏液性脂肪肉瘤(Myxoid liposarcoma,MLPS)/圆形细胞脂肪肉瘤(Round cell liposarcoma,RCLPS),以及发病机制复杂且多变的多形性脂肪肉瘤(Pleomorphic liposarcoma,PLPS)。ALT/WDLPS的主要病理变化为多形性成熟脂肪细胞增殖,DDLPS为高级别肉瘤,MLPS/RCLPS主要由非脂质间质细胞组成,而PLPS以异型多空泡脂肪母细胞为特点。这四个亚型所包含的肿瘤基因及临床病理学改变,导致了这四种亚型具有不同的临床行为、治疗敏感性和生物学特性。本文将对这四个亚型的病理及基因改变进行总结和探讨。Liposarcoma is a malignant tumor originating from primitive mesenchymal cells,which has remarkable tissue diversity and consists of cells with different degrees of differentiation and atypia.Liposarcoma is mainly divided into four subtypes:atypical lipogenic tumors(ALT)with MDM2-HMGA2 as the key gene/well-differentiated liposarcoma(WDLPS)and dedifferentiated liposarcoma(DDLPS),myxoid liposarcoma(MLPS)/round cell liposarcoma(RCLPS)with FUS-CHOP gene fusion as the main mechanism,and pleomorphic liposarcoma(PLPS)with complex and variable pathogenesis.The main pathological changes of ALT/WDLPS are the proliferation of pleomorphic mature adipocytes,DDLPS is high-grade sarcoma,MLPS/RCLPS is mainly composed of non-lipid stromal cells,and PLPS is characterized by atypical multivacuolar adipocytes.The tumor gene and clinicopathological changes contained in these four subtypes lead to the different clinical behaviors,treatment sensitivity and biological characteristics of these four subtypes.This paper summarizes and discusses the pathological and genetic changes of these four subtypes.

关 键 词：脂肪肉瘤 病理学 免疫组织化学 

分 类 号：R738.6[医药卫生—肿瘤]