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[脂肪瘤与同一肿瘤内的非典型脂肪瘤性肿瘤:连续转变的证据]

[Lipoma and atypical lipomatous tumor within the same neoplasia: Evidence for a continuous transition].

作者信息

Kuhnen C, Mentzel T, Lehnhardt M, Homann H H, Sciot R, Debiec-Rychter M

机构信息

Institut für Pathologie am Clemenshospital, Medical Center, Münster, Düesbergweg 128, 48153 Münster.

出版信息

Pathologe. 2010 Mar;31(2):129-34. doi: 10.1007/s00292-009-1257-6.

Abstract

The case of a lipomatous tumor with a predominant lipoma component and transition to an atypical lipomatous tumor is presented. A deep-seated soft tissue tumor of the right thigh with a maximum size of 14 cm was resected in a 70-year-old female patient. Corresponding to a comparable macroscopic aspect, the lesion revealed the histological features of an ordinary lipoma without atypia in about 80% of the specimen. In the remaining portion (approximately 20%) histopathology showed an atypical lipomatous tumor (ALT, lipoma-like subtype). Immunohistochemistry for MDM 2 and CDK4 revealed no immunoreactivity in the lipoma component, but within the ALT component. Interphase dual-color fluorescence in situ hybridization showed no amplification of the MDM 2 gene and rarely CDK4 gene amplification within the lipoma component, but high level amplification of MDM 2/CDK4 gene in the ALT area, further supporting the morphologically based diagnosis of a lipomatous tumor including areas of a true lipoma and ALT. This case underlines the concept of a continuous stepwise development of lipomatous soft tissue tumors from benign to malignant counterparts as a biological continuum.

摘要

本文报告一例以脂肪瘤成分为主并转变为非典型脂肪瘤性肿瘤的脂肪瘤样肿瘤病例。一名70岁女性患者,切除了位于右大腿的一个最大直径为14cm的深部软组织肿瘤。大体形态相似,病变在约80%的标本中显示为无异型性的普通脂肪瘤的组织学特征。在其余部分(约20%),组织病理学显示为非典型脂肪瘤性肿瘤(ALT,脂肪瘤样亚型)。MDM 2和CDK4的免疫组化显示在脂肪瘤成分中无免疫反应性,但在ALT成分中有免疫反应性。间期双色荧光原位杂交显示在脂肪瘤成分中MDM 2基因无扩增,CDK4基因很少扩增,但在ALT区域MDM 2/CDK4基因有高水平扩增,进一步支持了基于形态学的脂肪瘤样肿瘤诊断,包括真正脂肪瘤和ALT区域。该病例强调了脂肪瘤样软组织肿瘤从良性到恶性对应物的连续逐步发展作为一种生物学连续体的概念。

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