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浅表性CD34阳性纤维母细胞瘤的详细分析:一例报告并文献复习

Detailed analysis of a superficial CD34-positive fibroblastic tumor: A case report and review of the literature.

作者信息

Yamaga Kensaku, Fujita Akihiro, Osaki Mari, Kuwamoto Satoshi, Ishiguro Naoko, Yamamoto Tadahito, Nagashima Hideki

机构信息

Department of Orthopedic Surgery, Faculty of Medicine, Tottori University, Yonago, Tottori 683-8504, Japan.

Department of Pathology, Tottori University Hospital, Yonago, Tottori 683-8504, Japan.

出版信息

Oncol Lett. 2017 Sep;14(3):3395-3400. doi: 10.3892/ol.2017.6636. Epub 2017 Jul 20.

Abstract

Superficial cluster of differentiation (CD)34-positive fibroblastic tumor (SCPFT) is a rare mesenchymal neoplasm of borderline malignancy. It is characterized by a superficial location, marked cellular pleomorphism, an extremely low incidence of mitotic figures, and strong CD34 immunohistochemical positivity. As SCPFT is a recently described neoplasm, its characteristics are yet to be fully elucidated. To the best of our knowledge, no detailed studies regarding the imaging findings and cytogenetic analyses of SCPFTs exist. The present study describes a typical case of an 18-year-old man who developed an SCPFT measuring 87×70×80 mm in the subcutaneous adipose tissue of his right thigh. Computed tomography (CT) revealed a well-marginated tumor without calcification, and the enhancement on CT was weak. The tumor demonstrated abnormal uptake on 2-(18F) fluoro-2-deoxy-D-glucose positron emission tomography (PET), with a maximum standardized uptake value of 2.57. Magnetic resonance imaging (MRI) revealed a clearly defined tumor that exhibited homogeneous low signal intensity on T1-weighted imaging and high signal intensity on T2-weighted imaging, with small lobulated structures. Histopathologically, the tumor was composed of irregular spindle-to-oval-shaped cells with eosinophilic glassy cytoplasm and hyperchromatic, bizarre and pleomorphic nuclei that frequently exhibited intranuclear pseudoinclusions. Immunohistochemically, the tumor cells were diffusely and strongly positive for CD34. The Mindbomb E3 ubiquitin protein ligase 1 labeling index was 8.6%. Ultrastructurally, the tumor cells exhibited irregular or convoluted nuclei with abundant euchromatin-prominent nucleoli. The cytoplasmic organelles consisted of scattered, abundant rough endoplasmic reticulum, mitochondria, lysosomes, ribosomal rosettes and aggregated lipid globules. Of 18 metaphase cells identified, 2 demonstrated translocation between chromosomes 2 and 5 in cytogenetic studies. To the best of our knowledge, this is the first study describing imaging data (CT, MRI and PET-CT) and chromosomal aberrations for SCPFT.

摘要

浅表性分化簇(CD)34阳性成纤维细胞瘤(SCPFT)是一种罕见的交界性恶性间叶性肿瘤。其特点是位于浅表、细胞显著多形性、有丝分裂象发生率极低以及CD34免疫组化强阳性。由于SCPFT是一种最近才被描述的肿瘤,其特征尚未完全阐明。据我们所知,目前尚无关于SCPFT影像学表现和细胞遗传学分析的详细研究。本研究描述了一名18岁男性的典型病例,其右大腿皮下脂肪组织中出现了一个大小为87×70×80 mm的SCPFT。计算机断层扫描(CT)显示肿瘤边界清晰,无钙化,CT增强扫描呈轻度强化。该肿瘤在2-(18F)氟-2-脱氧-D-葡萄糖正电子发射断层扫描(PET)上显示异常摄取,最大标准化摄取值为2.57。磁共振成像(MRI)显示肿瘤边界清晰,在T1加权成像上呈均匀低信号强度,在T2加权成像上呈高信号强度,有小分叶结构。组织病理学上,肿瘤由不规则的梭形至椭圆形细胞组成,细胞质嗜酸性、呈玻璃样,细胞核深染、怪异且多形,常可见核内假包涵体。免疫组化显示,肿瘤细胞CD34弥漫性强阳性。Mindbomb E3泛素蛋白连接酶1标记指数为8.6%。超微结构上,肿瘤细胞核不规则或呈卷曲状,常染色质丰富,核仁明显。细胞质细胞器包括散在的丰富粗面内质网、线粒体、溶酶体、核糖体玫瑰花结和聚集的脂滴。在细胞遗传学研究中,在鉴定的18个中期细胞中,有2个显示2号和5号染色体之间发生了易位。据我们所知,这是第一项描述SCPFT影像学数据(CT、MRI和PET-CT)及染色体畸变的研究。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d5f2/5588033/9f0463124155/ol-14-03-3395-g00.jpg

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