CAJ | 학술논문

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。
목적：탐토원발척주우문육류（Ewing′s）/원시신경외배층종류（PNET）림상、병이학특정화영상학표현，제고대해류질병적인식。방법회고성분석남방의과대학제삼부속의원등6가의원2008년1월지2013년4월경병리증실적15례원발우척주저미골Ewing′s/PNET적림상、병리화영상자료，분석기영상표현특정。결과15례Ewing′s/PNET중，림상발병년령경，병정단；경단1례、흉단5례、요단2례、저미단7례。영상표현위불동정도골질파배（용골성파배9례，혼합형4례）화연조직종괴（배사、낭변13례，개화2례）。 CT평소표현위등혹저밀도，증강소묘균위불균균강화；MRI검사T1 WI상정저、등혹초고신호；T2 WI상균정불균균고혹초고신호；증강대다병례유명현강화。10례추체탑함、변형，각추간극균무협착혹증관、추간반신호무명현이상。상규HE염색，13례경하가견반유Homer-Wright （ H-W）“국형단”배렬。면역조화염색，신경표지물양성솔최고적시CD99，13례양성；기차시CD56、Ki67、Syn양성9례；Vim양성7례；NSE양성5례；S-100양성4례등。결론원발척주Ewing′s/PNET영상학표현위불동정도골질파배급연조직종괴영，밀도혹신호다불균균，다반유연착확대추간공향외생장등특점。단영상학표현결핍특이성，수결합림상발병년령、증상、진전정도등병사가제출진단，최후학진수조직병이학분석。
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 .