Angiofibroma of soft tissue
Angiofibroma of soft tissue
复制标题
软组织血管纤维瘤
DOI：
10.1097/01.pat.0000454061.00654.2f
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发表时间：
2014
期刊：
影响因子：
4.5
通讯作者：
Y. Oda
中科院分区：
文献类型：
--
作者：
Y. Oda
血管纤维瘤的软组织发生在大腿的一个54岁的女性将被证明。大体上，边界清楚的肿瘤呈灰白色，切面上有局灶性凝胶样外观。组织学上，肿瘤由椭圆形至短梭形细胞组成，排列不规则，伴有裂隙样或血管外皮细胞瘤样血管，纤维胶原或粘液样间质。免疫组织化学，肿瘤细胞是阴性的CD 34，肌原性，肌上皮或上皮标记。INI 1表达得以保留。RT-PCR检测到特征性AHRR-NCOA 2融合基因转录本。这种肿瘤的特征在于两个组成部分：纤维胶原或粘液样基质中均匀增殖的温和的梭形细胞，以及由小的、分支的血管外皮细胞瘤和薄壁血管组成的突出的血管网。在这张幻灯片中，我们将详细介绍软组织血管纤维瘤的临床病理特征。此外，这种特殊的肿瘤，包括细胞血管纤维瘤，孤立性纤维瘤，低度纤维粘液样肉瘤和低度粘液纤维肉瘤或粘液样/圆细胞脂肪肉瘤的鉴别诊断将被解释。
Angiofibroma of soft tissue arising in the thigh of a 54-year-old female will be demonstrated. Grossly, well circumscribed tumor showed grayish white color with focal gelatinous appearance on its cut surface. Histologically, the tumor is composed of oval to short spindle-shaped cells arranged in patternless pattern, accompanied by slit-like or hemangiopericytomatous vessels, fibro-collagenous or myxoid stroma. Immunohistochemically, tumor cells are negative for CD34, myogenic, myoepithelial or epithelial markers. INI1 expression is preserved. Characteristic AHRR-NCOA2 fusion transcript was detected by RT-PCR. This tumor is characterized by two components: uniformly proliferating bland spindle shaped cells in fibro-collagenous or myxoid stroma, and prominent vascular network composed of small, branching hemangiopericytomatous, and thin walled blood vessels. In this slide seminar detailed clinicopathological features of angiofibroma of soft tissue will be demonstrated. Moreover, the differential diagnosis of this peculiar tumor including cellular angiofibroma, solitary fibrous tumor, low-grade fibromyxoid sarcoma and low-grade myxofibrosarcoma or myxoid/round cell liposarcoma will be explained.