脂肪肉瘤的基因组学研究综述与评述

Hannah C. Beird, A. Lazar, Danh D. Truong
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摘要

软组织肉瘤是一种表现为间质和神经外胚层分化的恶性肿瘤,因此最常类似于支持性组织和结缔组织,包括脂肪、血管、肌肉、骨、肌腱和神经。肉瘤有70多种亚型,病理学家根据它们与分化细胞类型的相似程度对它们进行了广泛的分类专业脂肪细胞脂肪肉瘤的特点分为几个亚型发病率最高的四个welldifferentiated脂肪肉瘤WDLPS肉瘤脂肪肉瘤DDLPS黏液样脂肪肉瘤mlp和多形性脂肪肉瘤PLPS 1总体存活率最高mlp紧随其后WDLPS DDLPS然后PLPS2-4图2虽然WDLPS主要发生在四肢和腹膜后腔的深度软组织DDLPS来说位于腹膜后腔mlp PLPS尽管有这些广泛的分类,脂肪肉瘤也可以具有混合表型,并且通常进一步细分为具有其他超罕见特征的更罕见的实体,例如多形性MLPS具有PLPS和MLPS的属性6,7。
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The Genomics of Liposarcoma: A Review and Commentary
Soft tissue sarcomas STS are malignancies that show mesenchymal and neuroectodermal differentiation and thus most often resemble supportive and connective tissue including fat blood vessels muscle bone tendons and nerves Over 70 subtypes of sarcomas exist and pathologists have classified these broadly according to the degree to which they resemble differentiated cell types Figure 1 1 This review will focus on the most common subset of STS in adults liposarcoma which are tumors with histological features of specialized fat cells Liposarcoma are broken down into several subtypes The four with the highest incidence are welldifferentiated liposarcoma WDLPS dedifferentiated liposarcoma DDLPS myxoid liposarcoma MLPS and pleomorphic liposarcoma PLPS 1 Overall survival is highest for MLPS followed by WDLPS and DDLPS and then PLPS2-4 Figure 2 While WDLPS occurs predominantly in the deep soft tissues of the limbs and retroperitoneum DDLPS is located mostly in the retroperitoneum MLPS and PLPS are preferentially located within the limbs5 Despite these broad categories liposarcoma can also have mixed phenotypes and is often further subdivided into even more rare entities with other ultra-rare features For instance pleomorphic MLPS has attributes of both PLPS and MLPS6,7.
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