肠系膜韧带样型纤维瘤病CT及病理特征分析  

Analysis of CT and pathological features of mesenteric desmoid-type fibromatosis

作　　者：张海平 王凤丹 陈博 李爽 苏佰燕 薛华丹 张燕 杨正汉[1] 金征宇 ZHANG Haiping;WANG Fengdan;CHEN Bo;LI Shuang;SU Baiyan;XUE Huadan;ZHANG Yan;YANG Zhenghan;JIN Zhengyu(Department of Radiology,Beijing Friendship Hospital,Capital Medical University,Bingjing 100050,China;Department of Radiology,Peking Union Medical College Hospital,Peking Union Medical College and Chinese Academy of Medical Sciences,Beijing 100010,China;Department of Pathology,Peking Uniom Mdical College Hospital,Peking Union Medical College and Chinese Academy of Medical Sciences,Beijing 100010,China;Department of Magnetic Resonance Imaging,Fueai Hos pital,National Center for Cardioascular Diseases,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing 100037,China)

机构地区：[1]首都医科大学附属北京友谊医院放射科,北京100050 [2]中国医学科学院北京协和医学院北京协和医院放射科,北京100010 [3]中国医学科学院北京协和医学院北京协和医院病理科,北京100010 [4]中国医学科学院北京协和医学院国家心血管病中心阜外医院磁共振影像科,北京100037

出　　处：《实用放射学杂志》2021年第1期71-74,83,共5页Journal of Practical Radiology

摘　　要：目的总结肠系膜韧带样型纤维瘤病(DTF)的CT表现及病理特征.方法收集9例经手术病理证实的肠系膜DTF患者的临床资料、CT检查及病理结果.结果(1)肠系膜DTF的CT特征:①病灶均为单发软组织密度团块,呈圆形或类圆形,3例位于空肠系膜,4例位于回肠系膜,1例位于升结肠系膜,1例范围较广泛,位于空回肠及横结肠系膜,病灶最大径4.3~13.5cm,平均(7.9±3.0)cm;②CT平扫密度较均匀6例,密度不均匀3例,其中1例内见直径约1cm囊变区,2例病灶内部见点状脂肪密度,2例病灶边缘见点状脂肪密度影,1例病灶内见吻合线影,9例均无钙化;③CT增强扫描实性部分动脉期轻中度强化,静脉期进一步强化,延迟期持续强化,6例可见肠系膜上动脉分支供血,1例肠系膜上静脉受累,6例与邻近肠管局部分界不清.(2)肠系膜DTF病理学特征:大体标本显示病灶切面呈灰白/灰红色,8例实性、1例囊实性,质韧G硬.显微镜下,病灶内见大量梭形纤维母细胞、肌纤维母细胞及胶原纤维,细胞无异型,核分裂象较少见,另外镜下见7例病灶不同程度累及肠壁,3例累及肠壁浆膜层,1例累及肌层,3例累及肠壁全层.8例做了免疫组化染色检测,其中6/6例显示βGcatenin核阳性,5/5例显示Vimentin阳性,5/8例不同程度表达平滑肌肌动蛋白(SMA),仅1/7例部分表达Desmin,2/8例SG100散在+,8例均不表达CD117及DOGG1.(3)随访结果:7例获得随访,随访时间为3~20个月,平均(10.9±5.5)个月,随访期间均未见复发,1例为术后27个月后术区出现复发病灶,再次术后未再来院进行影像随诊.结论肠系膜DTF多为单发的圆形或类圆形软组织密度肿块,呈浸润性生长,可侵犯邻近肠壁,平扫病灶密度均匀或不均匀,无钙化,部分病灶内部或周边可见点状脂肪成分,少数可有囊变,增强扫描呈渐进性强化,强化程度在延迟期达顶峰,大部分病灶可见肠系膜上动脉分支供血.Objective To analyze and summarize the CT and pathological features of mesenteric desmoid-type fibromatosis(DTF).MethodsCinical data,CT findings and pathological findings in 9 cases of mescnteric DTF confirmed by pathology were collected.Results(1)CT findings of mesenteric DTF:①The lesions were single soft tissue density mass with round or similar-round shape.Three cases were located in the mesenteric of jejunum,4 in the mesenteric of ileum,1 in the mesenteric of ascending mesocolon and 1in the mesenteric of jejunum,iletm and transverse mesocolon.The maximum diameter of the lesion was 4.3-13,.5 cm,with an averagediameter of(7.9±3.0)cm.②CT plain scan showed homogeneous density in 6 cases and heterogeneous density in 3 ones.Amongthem,1 case had cystic degeneration about 1 cm in diameter,2 cases had focal fat density inside the lesion,2 cases had focal fat density in the edge of the lesion,1 case had concordant profile inside thelesion.All 9 cases had no calcification.③Enhanced scan of CT:The arterial phase was mild to moderate enhanced,and the venous phase was continuously enhanced,and the delayed lesion intensified to the peak.The branches of superior mesenteric artery were seenin 6 cases,the superior mesenteric vein was involved in 1 case,and 6 cases of lesions were not well defined with adjacent intestinaltracts.(2)Pathological features of mesenteric DTF:Gross specimen showed that lesions were solid in 8 cases and cyst-solid mixturein 1 case,tough to hard in quality,with greyish white or greyish red in section.Microscopically,a large number of spindle fibroblasts,myofibroblasts and colagen fibers were observed in the lesion.Cells were not heterotypic and caryokinesis were rare.The intestinalwall was involved in varying degrees under the microscopy in 7 cases,3 cases involved the serosa layer of the intestinal wall,1 case involvedthe muscular layer and 3 cases involved the whole intestinal wall.Ilmmunohistochemical staining was performed in 8 cases.Amongthem,6/6 expressed$-catenin nucleus,5/5 expressed Vim

关 键 词：肠系膜 韧带样型纤维瘤病 计算机体层成像 

分 类 号：R572.3[医药卫生—消化系统] R814.42[医药卫生—内科学]