软组织低度恶性纤维黏液样肉瘤的病理特征与MRI表现  

MRI and pathological findings of low-grade fibromyxoid sarcoma

作　　者：张燕绒 许崇永[2] 郑汉朋 邱乾德 

机构地区：[1]浙江省乐清市人民医院放射科,325600 [2]温州医科大学附属第二医院放射科 [3]浙江省温州市人民医院影像科

出　　处：《中华放射学杂志》2015年第12期889-894,共6页Chinese Journal of Radiology

摘　　要：目的探讨软组织低度恶性纤维黏液样肉瘤（LGFMS）MRI表现特征。方法回顾性分析经手术病理证实的13例LGFMS患者的MRI表现。对肿瘤的MRI信号、界限、囊变和强化特征与病理组织成分进行对照研究。结果13例患者，病灶位于大腿7例、小腿3例、肘部1例、肩部1例、上臂1例。MRI表现：肿瘤最大径2．5～12．0cm，平均（7．01±2．89）cm，其中〉5．0cm 11例。肿瘤界限均较清楚。其中瘤内坏死囊变5例，瘤内有分隔3例，瘤内见液-液平面2例，瘤周明显水肿3例。MRI T1WI序列呈低信号6例、等低信号4例，呈等信号、中心片状稍高信号2例，呈高低信号1例。T2WI高信号6例，其中中央见更高信号4例；呈高、等、低混杂信号4例；呈高、低信号3例。T2WI脂肪抑制呈高、等、低混杂信号8例；实质部分呈高信号，中央呈更高信号5例。增强均呈不均匀强化，其中轻度强化8例、中度强化4例、显著强化1例。病理检查：13例肉眼见肿物切面呈灰白色或灰黄色，边界清楚，质地硬、韧，其中5例可见囊性变，囊内见胶冻样物。镜下均见交替分布的纤维性和黏液性区域，其中9例有菊形团形成、2例有特征性的弓形血管。免疫组织化学5例均显示波形蛋白阳性，结蛋白、S-100阴性，其中3例肌动蛋白和CD34部分阳性。结论LGFMS具有一定的特征，MRI T1WI呈低、等信号，T2WI和T1WI脂肪抑制呈高、等、低混杂信号，瘤内液-液平面，结合临床提示LGFMS可能，确诊依靠病理检查。Objective To investigate the MRI characteristics of low grade fibromyxoid sarcoma （LGFMS）. Methods The MRI imaging of 13 cases with operation and pathology proven low grade fibromyxoid sarcoma were retrospectively analyzed. Comparative study of the relationship between the signal, boundary, cystic, and the enhancement degree with tumor tissue components was performed. Results The tumors were located in thigh （n=7）, lower leg （n=3）, elbow, shoulder and upper arm （n=1 for each）. The average diameter of tumor was （7.01±2.89） cm （range from 2.5 to 12.0 cm）. The tumors with maximal diameter larger than 5 cm were seen in 11 patients. All tumors had well-defined margin. Necrosis and cystic change was seen in 5 tumors, tumor compartment in 3 cases, intratumoral fluid fluid level in 2 cases, and peritumoral edema in 3 cases. T1WI showed hypointensity in 6 cases, iso-, hopointensity in 4 cases, isointensity with slightly hyperintensity in tumor center in 4 cases, heterogeneous intensity in 1 case. T2WI showed hyperintensity in 6 cases, central higher intensity in 4 cases, hyper-, iso-, hypo-intensity in 4 cases, and hyper-, hypo-intensity in 3 cases. T2WI with fat suppression sequence showed hyper-, iso-, hypo-intensity in 8 cases and high intensity solid part and much higher intensity in tumor center in 5 cases. Heterogeneous enhancement of the tumors was noted, including mild enhancement in 8 cases, moderate enhancement in 4 cases, and marked enhancement in one case, Pathological resuhs showed that the tumors were gray and white or yellow, well-defined, and hard texture, in which 5 cases cystic degeneration with tremelloid material can be seen in 5 cases. Microscopy results showed alternating distribution of fibrous and myxoid areas. Nine cases had rosettes formation and characteristic arcuate vessels were seen in 2 tumors. Immunohistochemical results showed negative Vimentin, Desmin, and S-100 in 5 patients, while 3 patients were positive for SMA and CD34. Conclusions LGFMS has some characterist

关 键 词：软组织肿瘤 磁共振成像 

分 类 号：R738.6[医药卫生—肿瘤] R445.2[医药卫生—临床医学]