骨脂肪硬化性黏液纤维性肿瘤10例临床病理诊断辨析及文献复习

doi:10.16150/j.1671-2870.2020.06.006

摘要/Abstract

摘要：

目的： 探讨骨脂肪硬化性黏液纤维性肿瘤（liposclerosing myxofibrous tumor, LSMFT）的临床表现、影像学和病理学特征。方法： 分析同济大学附属第十人民医院2015年5月至2019年间收治的10例LSMFT患者的临床表现、影像学资料及组织病理学特征,并复习相关文献。结果： 10例患者中,男性7例,女性3例,发病年龄为22~68岁,平均年龄为49岁。8例患者的肿瘤位于股骨近端或股骨粗隆间,2例患者的肿瘤位于胫骨近端。患者的临床症状无特异性,7例患者诉下肢局部疼痛、关节肿胀,3例患者无明显自觉症状,2例患者行走困难。患者的X线及CT图像表现为髓腔内偏心或沿股骨纵轴走向的地图样溶骨性破坏,病灶边界清晰、密度不均匀,具有硬化边缘。MRI显示T1加权像（T1 weighted image, T1WI）为相对均匀信号,T2加权像（T2 weighted image, T2WI）信号混杂,压脂序列有明显高信号。LSMFT的组织病理学特征多样,10例标本均呈现黏液纤维样区、纤维结构不良（fibrous dysplasia, FD）样骨和不规则钙化,7例标本有脂肪瘤样区,6例标本有Paget样骨,2例标本有泡沫样组织细胞聚集区,1例标本合并单纯性骨囊肿。10例患者均行病灶刮除术,随访1~60个月无复发。结论： LSMFT是一种好发于股骨近端的良性纤维骨性病变,黏液纤维样区、FD样骨和不规则钙化是其较为常见的组织学特点,应与FD、骨梗死等疾病进行鉴别。

关键词: 骨肿瘤, 脂肪硬化性纤维黏液样肿瘤, 纤维结构不良, 溶骨性病变, 病理学

Abstract:

Objective: To explore the radiological and clinicopathologic characteristics of liposclerosing myxofibrous tumor (LSMFT). Methods: Ten patients diagnosed with LSMFT were enrolled during May 2015 to 2019 in our hospital, and the data including the manifestations, imaging and pathological characteristics were studied retrospectively, and related literatures were reviewed. Results: There were 7 male and 3 female in 10 cases and the average age of onset was 49 years (22-68). All cases showed non-specific clinical symptoms, in which 7 cases complained local pain in lower limb and swollen joint and 2 cases had walking difficulty, while 3 cases didn't show any symptoms. As consistent with literatures, most lesions (8/10) located in the proximal femur or the intertrochanteric region, and 2 lesions were in the proximal tibia. The CT and X-ray images showed a geographic osteolytic lesion with intramedullary eccentric shape or along femur longitudinal axis. The lesions had clear edges, nonhomogeneous density or sclerotic margin. MRI showed the lesions had relatively homogeneous signal on T1WI, and heterogeneously high signal intensity on T2WI with fat suppression. Microscopically, LSMFT was characterized with diversely histologic pattern. The myxofibrous areas, bone trabeculae similar to fibrous dysplasia (FD) and irregular calcification were found in all cases (10/10), and lipomatous areas, Pagetoid bone, xanthoma cells and cyst formation were showed in 7/10, 6/10, 2/10, 1/10 cases, respectively. All patients were treated with curettage and no recurrence were reported during 1-60 month follow-up. Conclusions: LSMFT is a rare benign fibro-osseous lesion with unique radiological features and multiple pathological structures,characterized with myxofibrous areas, irregular calcification and bone trabecula similar to FD. LSMFT should be distinguished from FD and bone infarction.

Key words: Bone tumor, Liposclerosing myxofibrous tumor, Fibrous dysplasia, Lytic lesion, Pathology

中图分类号:

R739.96

金娇莺, 江潇, 徐昂, 张长宝, 李倩玉. 骨脂肪硬化性黏液纤维性肿瘤10例临床病理诊断辨析及文献复习[J]. 诊断学理论与实践, 2020, 19(06): 577-582.

JIN Jiaoying, JIANG Xiao, XU Ang, ZHANG Changbao, LI Qianyu. Liposclerosing myxofibrous tumor: clinicopathologic analysis of 10 cases and review of literature[J]. Journal of Diagnostics Concepts & Practice, 2020, 19(06): 577-582.

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参考文献 20

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病例	性别	年龄（岁）	部位	主诉及检查结果	病灶体积	影像学诊断
例1	女	68	股骨颈	外伤就诊,X线检查示右股骨颈骨髓腔内异常占位	2.8 cm×2.1 cm×1.6 cm	骨梗死
例2	男	22	股骨近端	左髋部扭伤9 d	9.3 cm×5.9 cm×3.3 cm	FD合并囊肿
例3	男	65	股骨近端	外伤就诊,X线检查发现右股骨粗隆间异常占位	4.0 cm×3.3 cm×2.4 cm	骨关节良性病变
例4	男	43	股骨近端	左髋疼痛2个月余	5.0 cm×2.9 cm×2.3 cm	骨关节良性病变
例5	男	58	股骨近端	左髋行走后酸胀,反复发作	5.0 cm×3.9 cm×3.2 cm	FD
例6	女	32	胫骨近端	无明显诱因下,右胫骨近端疼痛	3.4 cm×2.5 cm×1.6 cm	FD
例7	男	38	股骨颈	无明显诱因下,左髋关节肿胀	2.3 cm×2.1 cm×1.6 cm	FD
例8	男	42	股骨粗隆间	无明显诱因下,左髋部疼痛,反复发作	5.0 cm×4.6 cm×2.9 cm	FD
例9	女	51	股骨转子间	因腰背部疼痛、腰椎间盘突出,行腰椎摄片及骨盆摄片后,发现右股骨骨髓腔内病灶	5.0 cm×3.5 cm×1.0 cm	骨良性肿瘤
例10	男	68	胫骨近端	无明显诱因下,出现右膝疼痛, 右侧膝关节伸屈活动受限	4.1 cm×3.7 cm×2.1 cm	脂肪瘤