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目的 探讨骨盆原发尤文氏肉瘤的临床、病理学特征和影像学表现,提高对该病的认识和诊断率.方法 回顾性分析2008年5月至2016年3月杭州师范大学附属医院、河北医科大学第三医院及河北医科大学第二医院收治并经手术病理证实的13例骨盆原发尤文氏肉瘤的临床、病理及影像学资料,总结其特征.结果 13例骨盆原发尤文氏肉瘤发病中位年龄17岁.X线、CT表现为溶骨性及混合性骨质破坏,CT示混合型10例,8例肿瘤内骨化为絮状,10例见骨质膨胀性破坏,10例见骨膜反应,其中层状5例,放射状5例.13例均见软组织肿块影,呈等或稍低密度.4例增强扫描不均匀强化.磁共振图像(MRI)示病变T1WI低信号,T2WI混杂高信号,边界模糊,5例见斑片状坏死区,9例见不完整的假包膜,周围软组织局部受侵.4例MRI增强扫描呈不均匀强化.结论 骨盆尤文氏肉瘤好发于青少年男性,以混合型骨质破坏为主,软组织肿块较大,骨膜反应以层状及放射状为主,多数病例见不完整的假包膜.结合临床及影像学检查,可以对该病做出诊断.

Objective To explore the clinical,pathological and imaging features of Ewing's sarcoma in pelvis and to improve knowledge and diagnosis of the disease.Methods A retrospective analysis of the clinical,pathological and imaging data of pathologically confirmed 13 cases of Ewing's sarcoma in pelvis was carried out between May 2008 and March 2016 in the Affiliated Hospital of Hangzhou Normal University,the Third Hospital of Hebei Medical University and the Second Hospital of Hebei Medical University.Results The median age 13 cases of pelvic primary Ewing's sarcoma was 17 years old.The X-ray and CT imagings showed osteolytic and mixed bone destruction,CT showed mixed type in 10 cases,8 cases of bone tumors as a flocculent,10 cases of bone expansion failure,10 cases of periosteal reaction,the layered 5 cases,radial in 5 cases.Thirteen cases showed soft tissue mass,soft tissue mass was equal or slightly lower density.Four cases showed heterogeneous contrast enhancement.The lesions showed low signal in T1 WI and mixed high signal in T2WI of magnetic resonance imaging(MRI).The boundary of the lesions were obscure,and 5 cases had patchy necrosis area,and 9 cases had incomplete false capsule,surrounding soft tissue was violated.Four cases showed heterogeneous contrast enhancement after MRI enhancement scan.Conclusions The age of onset of Ewing's sarcoma of the pelvis is more concentrated in about 15 years.The imaging feaures are mixed bone destruction and more bone is swelling and permeability damage,soft tissue mass is larger,bone tumor is cloudy or acicular,periosteal reaction in a layered and radial,most cases show that the false envelope is not complete.Combined with clinical and imaging examination,the diagnosis of the disease can be made.