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血管肌纤维母细胞瘤的脂肪瘤样变型:2例报告并文献复习

Lipomatous variant of angiomyofibroblastoma: report of two cases and review of the literature.

作者信息

Cao Dengfeng, Srodon Monica, Montgomery Elizabeth A, Kurman Robert J

机构信息

Department of Pathology, The Johns Hopkins Hospital, Baltimore, Maryland 21287, USA.

出版信息

Int J Gynecol Pathol. 2005 Apr;24(2):196-200. doi: 10.1097/01.pgp.0000156765.90520.23.

Abstract

Angiomyofibroblastoma (AMF) is a rare, benign, mesenchymal tumor occurring mainly in the female genital tract. Some cases contain scattered mature adipocytes, but the lipomatous variant in which mature adipose tissue is prominent or striking is rare. Only five cases have been reported in the English literature. We report two more such cases that were composed of 70 to 80% and 30 to 40% adipose tissue, respectively. Immunohistochemical analysis showed that the tumor cells were positive for estrogen receptor, progesterone receptor, vimentin, and Bcl-2, and negative for cytokeratin AE1/1, EMA, and CD117. Tumor cells in the first case were positive for CD34 but not desmin and muscle-specific actin. The opposite expression profile of these three markers was observed in tumor cells in the second case: positive for desmin and muscle-specific actin and negative for CD34. Rare cells were positive for S-100 in adipose-rich areas in the first case. Our findings indicate that the tumor cells in the lipomatous variant have similar immunoprofile to those of usual AMF and support the concept that the lipomatous variant probably represents an extreme end of a wide spectrum of differentiation in AMF.

摘要

血管肌成纤维细胞瘤(AMF)是一种罕见的良性间叶组织肿瘤,主要发生于女性生殖道。部分病例含有散在的成熟脂肪细胞,但成熟脂肪组织显著或突出的脂肪瘤样变型较为罕见。英文文献中仅报道过5例。我们报告另外2例这样的病例,分别由70%至80%和30%至40%的脂肪组织构成。免疫组织化学分析显示,肿瘤细胞雌激素受体、孕激素受体、波形蛋白和Bcl-2呈阳性,细胞角蛋白AE1/1、上皮膜抗原(EMA)和CD117呈阴性。第一例病例中的肿瘤细胞CD34呈阳性,但结蛋白和肌特异性肌动蛋白呈阴性。在第二例病例的肿瘤细胞中观察到这三种标志物的表达情况相反:结蛋白和肌特异性肌动蛋白呈阳性,CD34呈阴性。在第一例病例富含脂肪的区域,罕见细胞S-100呈阳性。我们的研究结果表明,脂肪瘤样变型中的肿瘤细胞与普通AMF的肿瘤细胞具有相似的免疫表型,并支持脂肪瘤样变型可能代表AMF广泛分化谱的极端情况这一概念。

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