Dodd Leslie G
Department of Pathology, Duke University Medical Center, Durham, North Carolina 27710-3712, USA.
Diagn Cytopathol. 2012 Dec;40(12):1122-31. doi: 10.1002/dc.21794. Epub 2011 Sep 19.
Liposarcoma (LS) represents the most common type of soft tissue sarcoma. LS is subdivided into four distinct subtypes: well-differentiated, myxoid/round cell, pleomorphic, and mixed LS. In addition to distinctive morphology, each of the subgroups has a different prognosis and treatment strategy. While subgrouping has traditionally been based on conventional morphology, recent molecular and cytogenetic findings have validated the current classification system. Well-differentiated LS is characterized by overexpression of MDM2, which blocks the tumor suppressor function of p53. Myxoid/round cell LS is characterized by a specific translocation resulting in a fusion protein which interferes with adipocyte differentiation. Pleomorphic LS, like other high grade sarcomas, is characterized by multiple chromosomal abnormalities. Each of these distinctive molecular signatures can be exploited to aid in the subtyping of LS and in distinguishing true sarcomas of lipogenic derivation from benign mimics.
脂肪肉瘤(LS)是最常见的软组织肉瘤类型。LS可细分为四种不同的亚型:高分化型、黏液样/圆形细胞型、多形性和混合型LS。除了独特的形态学特征外,每个亚组还有不同的预后和治疗策略。虽然传统上亚组划分是基于传统形态学,但最近的分子和细胞遗传学研究结果证实了当前的分类系统。高分化型LS的特征是MDM2过表达,其阻断了p53的肿瘤抑制功能。黏液样/圆形细胞型LS的特征是特定的易位,导致一种融合蛋白干扰脂肪细胞分化。多形性LS与其他高级别肉瘤一样,其特征是存在多种染色体异常。这些独特的分子特征中的每一个都可用于辅助LS的亚型分类,并将真正的脂肪源性肉瘤与良性模仿物区分开来。