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非典型脂肪瘤:眼眶内具有去分化区域的高分化脂肪肉瘤。

Atypical lipoma: well-differentiated liposarcoma of the orbit with dedifferentiated areas.

作者信息

Stiglmayer Neda, Jandrokovicć Sonja, Miklicć Pavle, Hutinec Zdenka

机构信息

Department of Ophthalmology, Zagreb University School of Medicine, Zagreb, Croatia.

出版信息

Orbit. 2003 Dec;22(4):311-6. doi: 10.1076/orbi.22.4.311.17247.

Abstract

Primary orbital liposarcoma is a very rare orbital tumor and dedifferentiation in the liposarcomas occurs rarely. Dedifferentiation is defined by development, in a well-differentiated tumor, of poorly differentiated, high-grade areas. The dedifferentiated liposarcomas (DDLS) have a better prognosis than pleomorphic sarcomas as a whole. The treatment of DDLS patients proves to be complicated due to the fact that the histopathological diagnosis usually becomes evident only after the surgery. In the present paper, the clinical presentation, histopathological features, radiological characteristics and clinical treatment of one case of well-differentiated liposarcoma with dedifferentiated areas, abruptly delimited from the well-differentiated adipocytic component, are reported.

摘要

原发性眼眶脂肪肉瘤是一种非常罕见的眼眶肿瘤,脂肪肉瘤中的去分化现象很少发生。去分化是指在一个高分化肿瘤中出现低分化、高级别区域。总体而言,去分化脂肪肉瘤(DDLS)的预后比多形性肉瘤要好。由于组织病理学诊断通常仅在手术后才变得明显,因此DDLS患者的治疗颇具复杂性。本文报告了一例具有去分化区域的高分化脂肪肉瘤病例,其去分化区域与高分化脂肪细胞成分界限分明,介绍了该病例的临床表现、组织病理学特征、放射学特点及临床治疗情况。

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