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软组织孤立性纤维性肿瘤:15例报告,包括5例恶性病例,并附有光镜、免疫组化及超微结构资料

Solitary fibrous tumor of soft tissue: a report of 15 cases, including 5 malignant examples with light microscopic, immunohistochemical, and ultrastructural data.

作者信息

Nielsen G P, O'Connell J X, Dickersin G R, Rosenberg A E

机构信息

The James Homer Wright Pathology Laboratories, Massachusetts General Hospital, Harvard Medical School, Boston 02114, USA.

出版信息

Mod Pathol. 1997 Oct;10(10):1028-37.

PMID:9346183
Abstract

We describe 15 soft tissue solitary fibrous tumors (SFTs) occurring in patients 24 to 78 years old (average, 50.6 yr). Ten tumors were benign and arose in the head and neck area (three tumors), thigh (two), vulva (two), upper arm (one), lower leg (one), and retroperitoneum (one). Five tumors were histologically malignant and arose in the thigh (two), abdominal wall (one), buttock (one), and retroperitoneum (one). All of the tumors were grossly well circumscribed. The benign tumors measured from 2 to 10 cm (average, 4.8 cm) and the malignant ones from 3 to 5.5 cm (average, 4.3 cm) in greatest diameter. Microscopically, the benign tumors showed areas of hypercellularity with variable amounts of collagenous and myxoid stroma; one had amianthoid fibers. The malignant tumors were composed of cytologically atypical cells enmeshed in a collagenous or myxoid extracellular matrix. Ultrastructural study of three benign and three malignant tumors showed fibroblastic differentiation; one benign tumor showed myofibroblastic differentiation. Immunohistochemically, all of the tumors examined were immunoreactive for vimentin, and seven of nine were positive for CD34, including all of the malignant ones. There was focal staining for muscle actin in two benign tumors and for Leu-7 in one benign tumor; there was no staining for cytokeratin, desmin, S-100 protein, epithelial membrane antigen, or smooth muscle actin in any of the examined tissues. Follow-up was available for eight patients for 6 to 21 months (average, 12 mo). No tumor recurred locally or metastasized. The SFTs reported herein support the experiences of others who recently described these tumors in the somatic soft tissues. In addition, our series highlights the occurrence of malignant SFTs in the soft tissues. SFTs should be separated from other spindle cell sarcomas, with which they can be confused.

摘要

我们描述了15例发生于24至78岁(平均50.6岁)患者的软组织孤立性纤维性肿瘤(SFT)。10例肿瘤为良性,发生于头颈部区域(3例)、大腿(2例)、外阴(2例)、上臂(1例)、小腿(1例)和腹膜后(1例)。5例肿瘤组织学上为恶性,发生于大腿(2例)、腹壁(1例)、臀部(1例)和腹膜后(1例)。所有肿瘤大体上边界清楚。良性肿瘤最大直径为2至10 cm(平均4.8 cm),恶性肿瘤最大直径为3至5.5 cm(平均4.3 cm)。显微镜下,良性肿瘤显示细胞增多区域,伴有数量不等的胶原性和黏液样间质;1例有石棉样纤维。恶性肿瘤由陷入胶原性或黏液样细胞外基质中的细胞学上非典型细胞组成。对3例良性和3例恶性肿瘤的超微结构研究显示有成纤维细胞分化;1例良性肿瘤显示肌成纤维细胞分化。免疫组织化学检查,所有检测的肿瘤波形蛋白均呈免疫反应性,9例中的7例CD34呈阳性,包括所有恶性肿瘤。2例良性肿瘤肌动蛋白呈局灶性染色,1例良性肿瘤Leu-7呈阳性;在所检测的任何组织中,细胞角蛋白、结蛋白、S-100蛋白、上皮膜抗原或平滑肌肌动蛋白均无染色。8例患者获得了6至21个月(平均12个月)的随访。无肿瘤局部复发或转移。本文报道的SFT支持了其他人最近在躯体软组织中描述这些肿瘤的经验。此外,我们的系列病例突出了软组织中恶性SFT的发生情况。SFT应与其他可能与之混淆的梭形细胞肉瘤相鉴别。

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