骨硬化性上皮样纤维肉瘤影像表现一例
- Author:
- No author available
- Journal Title:
- Chinese Journal of Radiology
- Issue:
- 10
- DOI:
- 10.3760/cma.j.issn.1005-1201.2017.10.019
- Key Word:
- No keyword available
Abstract： 患者 男,30岁.因"发现左肘部肿块伴活动后酸胀数年"入院.患者数年前发现左肘部硬性固定肿块,活动后自觉左肘部酸胀不适感,至当地医院就诊,X线检查示左尺骨骨肿瘤,具体不详,未予处理.患者近期自觉左肘部肿块明显变大,不适感明显,3个月余前至我院门诊就诊.体格检查示:左肘部背侧鹰嘴下方约6.0 cm×4.0 cm×1.5 cm皮下隆起,质硬、固定、边界欠清、无压痛.肘关节活动范围30°~120°,伸肘稍受限.实验室检查血常规未见明显异常.左肘关节CT示左侧尺骨近端虫噬样骨质破坏及云絮状成骨性改变,骨质破坏区亦可见斑片状高密度影,周围见软组织肿块影,未见明显骨膜增生改变,病灶范围约6.9 cm × 4.8 cm,高密度区CT值约78 HU,软组织肿块区CT值约45 HU (图1).MRI示左尺骨近段团片状异常信号影,约6.9 cm× 5.8 cm×4.9 cm,T1WI上以等低信号为主(图2).压脂T2WI呈高低混杂信号(图3),尺骨近端骨质破坏.压脂T1WI增强扫描病灶周边见环形强化(图4),周围组织呈受压改变.手术所见:于左肘关节后内侧作长约15.0 cm切口,显露左尺骨近段及鹰嘴,尺骨近段至鹰嘴皮质破坏,有约10.0 cm× 8.0 cm×5.0 cm肿块占据.肿块边界清晰、质地韧,予以完整切除.切取肿瘤组织病理检查:左尺骨近端组织常规病理,显微镜下可见梭形细胞伴中央大片退变坏死钙化,核分裂活跃,肿瘤细胞侵及周边肌肉组织(图5).免疫病理:b-链蛋白(b-Catenin)、结蛋白(Desmin)、肌动蛋白(SMA)、白细胞表面分化抗原(CD)56、S-100、MBP、CD34、B淋巴细胞-2 (Bcl-2)均为阴性,细胞增殖指数(Ki-67)30%~60%,考虑为硬化性上皮样纤维肉瘤(sclerosing epithelioid fibrosarcoma, SEF).
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