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低度恶性纤维黏液样肉瘤:病例报告及免疫组化研究

Low-grade fibromyxoid sarcoma: case report and immunohistochemical study.

作者信息

Nichols G E, Cooper P H

机构信息

Department of Pathology, University of Virginia Health Sciences Center, Charlottesville 22908.

出版信息

J Cutan Pathol. 1994 Aug;21(4):356-62. doi: 10.1111/j.1600-0560.1994.tb00712.x.

Abstract

A case is presented of low-grade fibromyxoid sarcoma involving the arm of a 52-year-old man. Low-grade fibromyxoid sarcoma is a recently described neoplasm of the deep and subcutaneous soft tissue which demonstrates a spectrum of histologic images. The current case demonstrated the typical patterns of intermixed, sweeping bands of fibrous and myxoid tissue, homogeneous foci of fibrous and myxoid tissue, focal areas of storiforming, and concentric perivascular cuffs of slender spindle cells, all lacking the nuclear anaplasia, mitotic activity, and necrosis generally associated with sarcoma. Immunohistochemical analysis performed on paraffin-embedded sections demonstrated strong labeling of the tumor cells by anti-CD34 antibody, moderate labeling for vimentin, and rare, focal positivity for muscle-specific actin. Tumor cells were negative for markers of epithelial, muscular, neural, histiocytic, melanocytic, and vascular differentiation. The constellation of histopathologic features described in this and previous reports is characteristic of low-grade fibromyxoid sarcoma. Based on this case, it appears that the immunohistochemical features of low-grade fibromyxoid sarcoma can help to exclude many cutaneous and deep soft tissue tumors from the differential diagnosis. The findings support the interpretation that the neoplasm is essentially fibroblastic in nature.

摘要

本文报告一例发生于一名52岁男性手臂的低度纤维黏液样肉瘤。低度纤维黏液样肉瘤是一种最近才被描述的发生于深部和皮下软组织的肿瘤,具有一系列组织学表现。本例显示出典型的组织学形态,包括纤维组织和黏液样组织混合的、呈交织状的条带,纤维组织和黏液样组织的均匀区域,漩涡状形成的局灶性区域,以及细长梭形细胞围绕血管呈同心环状排列,所有这些均缺乏通常与肉瘤相关的核异型性、有丝分裂活性和坏死。对石蜡包埋切片进行免疫组织化学分析显示,肿瘤细胞被抗CD34抗体强烈标记,波形蛋白呈中度标记,肌特异性肌动蛋白呈罕见的局灶性阳性。肿瘤细胞对上皮、肌肉、神经、组织细胞、黑素细胞和血管分化的标志物均为阴性。本病例及既往报告中所描述的组织病理学特征组合是低度纤维黏液样肉瘤的特征。基于本病例,低度纤维黏液样肉瘤的免疫组织化学特征似乎有助于在鉴别诊断中排除许多皮肤和深部软组织肿瘤。这些发现支持该肿瘤本质上为成纤维细胞性的解释。

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