摘要
目的：探讨原发脊柱尤文肉瘤（Ewing′s）/原始神经外胚层肿瘤（PNET）临床、病理学特征和影像学表现，提高对该类疾病的认识。方法回顾性分析南方医科大学第三附属医院等6家医院2008年1月至2013年4月经病理证实的15例原发于脊柱骶尾骨Ewing′s/PNET的临床、病理和影像资料，分析其影像表现特征。结果15例Ewing′s/PNET中，临床发病年龄轻，病程短；颈段1例、胸段5例、腰段2例、骶尾段7例。影像表现为不同程度骨质破坏（溶骨性破坏9例，混合型4例）和软组织肿块（坏死、囊变13例，钙化2例）。 CT平扫表现为等或低密度，增强扫描均为不均匀强化；MRI检查T1 WI上呈低、等或稍高信号；T2 WI上均呈不均匀高或稍高信号；增强大多病例有明显强化。10例椎体塌陷、变形，各椎间隙均无狭窄或增宽、椎间盘信号无明显异常。常规HE染色，13例镜下可见伴有Homer-Wright （ H-W）“菊形团”排列。免疫组化染色，神经标志物阳性率最高的是CD99，13例阳性；其次是CD56、Ki67、Syn阳性9例；Vim阳性7例；NSE阳性5例；S-100阳性4例等。结论原发脊柱Ewing′s/PNET影像学表现为不同程度骨质破坏及软组织肿块影，密度或信号多不均匀，多伴有沿着扩大椎间孔向外生长等特点。但影像学表现缺乏特异性，需结合临床发病年龄、症状、进展程度等病史可提出诊断，最后确诊需组织病理学分析。
Objective To explore the clinicopathological and imaging features of Ewing′s sarcoma/peripheral primitive neuroectodermal tumors in vertebral column and to improve knowledge of the disease .Methods The imaging information of 15 cases with pathologically proved.Ewing′s/PNET were reviewed retrospectively.Results There was an earlier onset with a short disease course.The locations of tumor included cervical segment （n=1）, thoracic segment （n=5）, lumbar segment （n=2） and sacrococcyyeal region （n=7）.The imaging showed different extents of bone destruction.Among them, on radiology and computed tomography （CT）, osteolytic （n=9） and mixed （n=4） destruction were detected along with large soft tissue masses （ cystic and necrotic , n=13; calcification , n=2 ）.Isodensity or low density was shown on CT , heterogeneous contrast enhancement of hypointensity or isointensity or slight hyperintensity on T1WI and slight hyperintensity or hyperintensity on T 2WI of magnetic resonance imaging （ MRI）.Most cases showed obvious strengthening.Ten cases showed a collapse of centrum vertebrae while intervertebral space and disc had no change.All cases grew outside across intervertebral foramina.Hematoxylin-eosin staining demonstrated Homer-Wright （ H-W） rosettes in 13 cases.Immunohistochemistry , the highest positive rate of neural markers was CD99 （n=13）;secondly, CD56 and Ki67 and Syn （n=9）;Vim （n=7）, NSE （n=5） and S-100 （ n =4）, etc.Conclusion The non-specific imaging findings of Ewing′s/PNET show different extents of bone destruction in vertebral column and soft tissue masses outside across intervertebral foramina.Other clinical features and histopathology must be considered for making a definite diagnosis .
出处
《中华医学杂志》
CAS
CSCD
北大核心
2014年第23期1808-1811,共4页
National Medical Journal of China
关键词
肉瘤
Ewing
外胚层
磁共振成像
Sarcoma, Ewing＇s
Ectoderm
Magnetic resonance imaging
