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非典型梭形细胞/多形性脂肪肉瘤。

Atypical spindle cell/pleomorphic lipomatous tumor.

机构信息

Department of Pathology, Ghent University and Ghent University Hospital, Ghent, Belgium.

Cancer Research Institute Ghent (CRIG), Ghent University and Ghent University Hospital, Ghent, Belgium.

出版信息

Histol Histopathol. 2020 Aug;35(8):769-778. doi: 10.14670/HH-18-210. Epub 2020 Feb 18.

Abstract

Atypical spindle cell/pleomorphic lipomatous tumor (ASPLT) is a recently described morphologically low-grade and clinically indolent adipocytic tumor, which will be incorporated as a new tumor entity in the upcoming 5th edition of the WHO Classification of Soft tissue and Bone tumors. Histologically, ASPLTs are characterized by ill-defined tumor margins and the presence of variable proportions of mild-to-moderately atypical spindle cells, adipocytes, lipoblasts, pleomorphic multinucleated cells and a myxoid or collagenous extracellular matrix. ASPLTs can show a wide variety of microscopic appearances and there is histologic overlap with diverse mimics. The diagnosis of ASPLT can therefore be challenging. Molecular studies have shown a consistent absence of MDM2 or CDK4 amplification. On the other hand, deletions or losses of 13q14, including RB1, have been identified in a significant subset of cases. This review provides an overview of the currently known clinical and pathological features of ASPLTs, detailing its most relevant differential diagnoses.

摘要

非典型性梭形/多形性脂肪肉瘤(ASPLT)是一种新近描述的形态学低度恶性和临床惰性的脂肪细胞肿瘤,将被纳入即将出版的第 5 版世界卫生组织软组织和骨肿瘤分类中的新肿瘤实体。组织学上,ASPLTs 的特点是肿瘤边界不清晰,存在不同比例的轻度至中度非典型梭形细胞、脂肪细胞、脂肪母细胞、多形性多核细胞和黏液样或胶原性细胞外基质。ASPLTs 可以表现出各种各样的微观表现,与多种模拟物存在组织学重叠。因此,ASPLT 的诊断具有一定挑战性。分子研究表明,MDM2 或 CDK4 扩增缺失一致。另一方面,在相当一部分病例中发现了 13q14 的缺失或丢失,包括 RB1。这篇综述概述了目前已知的 ASPLT 的临床和病理特征,详细说明了其最相关的鉴别诊断。

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