腹盆腔促纤维组织增生性小圆细胞瘤的临床、影像学和病理学特征分析  

Clinical, imaging and pathological features of desmoplastic small round cell tumor in abdominal and pelvic cavity

作　　者：胡冠男 周良平 周正荣 张盛箭 HU Guannan;ZHOU Liangping;ZHOU Zhengrong;ZHANG Shengjian(Department of Radiology,Minhang District Branch of Cancer Hospital,Fudan University,Shanghai 200240,China;Department of Imaging,Xuhui Kangjian Street Health Service Center,Shanghai 200030,China;Department of Radiology,Fudan University Shanghai Cancer Center,Department of Oncology,Shanghai Medical College,Fudan University,Shanghai 200032,China)

机构地区：[1]复旦大学附属肿瘤医院闵行分院放射科,上海200240 [2]徐汇区康健街道社区卫生服务中心影像科,上海200030 [3]复旦大学附属肿瘤医院放射诊断科,复旦大学上海医学院肿瘤学系,上海200032

出　　处：《肿瘤影像学》2022年第3期309-315,共7页Oncoradiology

摘　　要：目的:探讨促结缔组织增生性小圆细胞肿瘤(desmoplastic small round cell tumor,DSRCT)的临床、影像学及病理学特征。方法:回顾并分析经复旦大学肿瘤医院病理学检查证实的30例DSRCT患者的临床资料、影像学及病理学特特征,并对其表现进行特征性分析。结果:30例患者中男性22例,女性8例,发病年龄17~49岁,中位年龄32岁。临床首发症状为腹部肿块11例(36.7%),30例患者中20例为首发患者,9例为术后复发,1例仅肿块切除术后未随访。此29例中,因1例患者以正电子发射体层成像(positron emission tomography,PET)为首诊方式,故选取28例中有计算机体层成像(computed tomography,CT)或磁共振成像(magnetic resonance imaging,MRI)影像学资料的患者进行评估,CT及MRI检查肿块分布于大网膜22例(78.6%)、小网膜12例(42.9%)、系膜间隙13例(46.4%);累及脏器包膜者,肝包膜下17例(60.7%)、脾包膜下6例(21.4%),其他脏器包膜6例(21.4%)。28例患者中有25例行CT增强检查,13例患者肿块内出现液化坏死(52.0%),6例患者肿块内出现钙化灶(24.0%)。肿块液化坏死区CT值为-4.9~40.5 HU(平均值17.8 HU),实性成分增强扫描前后CT值分别为17.8~41.6 HU(平均值38.6HU)及41.6~105.4 HU(平均值7 3.5H U);6例患者行M R I检查,腹盆腔无明显脏器起源多发结节及肿块影,以网膜系膜居多,T 1加权成像(T1-weighted imaging)呈等低信号,T2加权成像(T2-weighted imaging)呈混杂高信号,液化坏死呈高信号,弥散加权成像(diffusion-weighted imaging,DWI)呈高信号,动态增强呈轻中度不均匀强化。免疫组织化学分析显示弥漫强阳性表达广谱细胞角蛋白AE1/AE3(76.7%)、波形蛋白Vimentin(56.7%)及结蛋白Desmin(76.7%)。结论:DSRCT好发于青年男性,影像学检查多表现为腹腔网膜系膜或脏器间隙内多发弥漫性的肿块、膀胱直肠间隙肿块,无明确脏器起源,可囊变坏死、钙化,增强扫描呈轻中度不均匀延迟强化。临床、影像学�Objective: To investigate the clinical, imaging and pathological features of desmoplastic small round cell tumor(DSRCT). Methods: The clinical data, imaging and pathological features of 30 DSRCT patients confirmed by pathology in Fudan University Cancer Hospital were retrospectively analyzed, and their manifestations were characterized. Results: Among the 30 cases,22 were male and 8 were female, Age 17-49 years, and the median age was 32 years. The first clinical symptom was abdominal mass in 11 cases(36.7%). Of the 30 patients, 20 were first-time patients, 9 cases were postoperative recurrence, and 1 case was not followed up after mass resection. Of the 29 cases, because one patient took positron emission tomography(PET) as the first diagnosis. Therefore, 28 patients with computed tomography(CT) or magnetic resonance imaging(MRI) imaging data were selected for evaluation. CT and MRI showed that the mass was located in the greater omentum in 22 cases(78.6%), the lesser omentum in 12 cases(42.9%), and the mesenteric space in 13 cases(46.4%). Among the involved organ envelopes, 17 cases(60.7%) were subhepatic, 6 cases(21.4%) were subsplenic, and 6 cases(21.4%) were other organ envelopes. 25 of 28 patients underwent CT enhancement, liquefaction necrosis in 13 cases(52.0%), calcification in 6 patients(24.0%). The CT value of the liquefied and necrotic area of the mass was-4.9-40.5 HU(average 17.8 HU), and the CT values of the solid component before and after enhancement were 17.8-41.6 HU(average 38.6 HU) and 41.6-105.4 HU(average 73.5 HU). Six patients underwent MRI examination, and the abdominal and pelvic cavity showed no obvious organ origin, multiple nodules and mass shadows, mostly the mesentery. T1-weighted imaging(T1WI) showed equal low signal;T2-weighted imaging(T2WI) showed mixed high signal;liquefied necrosis showed high signal;diffusion-weighted imaging(DWI) showed high signal;dynamic enhancement showed mild to moderate heterogeneous enhancement. Diffuse strong positive expression of broad-spectrum cytoker

关 键 词：促结缔组织增生性小圆细胞肿瘤 计算机体层成像 磁共振成像 

分 类 号：R730.26[医药卫生—肿瘤] R445.2[医药卫生—临床医学] R445.3