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目的探讨Nora病的影像、病理学特征及鉴别诊断,以期提高对本病的认识及诊断水平。方法回顾性分析经手术及病理证实的5例Nora病的影像学表现并复习相关文献。由影像科医师及病理科医师共同阅片,将影像学与病理学结果进行对照分析。结果 5例Nora病均因发现局限性包块就诊,X线及CT均表现为骨旁斑片状欠均匀高密度影,边界清楚,病变与附着骨间骨髓腔不相连续,3例病变基底部存在低密度间隙。1例病变表面于MRI上见“软骨帽”样长T2信号。镜下表现为5例病变由分化成熟的软骨、骨和纤维组织3种成分无序排列,软骨形成帽状结构及掺杂在其他两种成分中,部分软骨细胞表现轻度异型性,骨基质蓝染表现为特征性的“蓝骨”。结论有助于Nora病的影像诊断指标为:(1)骨旁不规则结节灶;(2)病变直径多<30 mm;(3)X线及CT上病灶表现为欠均匀高密度结节;(4)X线及CT上病灶与附着骨间低密度间隙;(5)MRI上病变表面“软骨帽”样长T2信号。最终确诊仍需病理组织学检查。
Objective To investigate the imaging, pathological features and differential diagnosis of Nora disease in order to improve the understanding and diagnosis of this disease. Methods We retrospectively analyzed the imaging findings of 5 cases of Nora disease confirmed by surgery and pathology and reviewed the related literatures. By the imaging physicians and pathologists to read the film, the imaging and pathology results were analyzed. Results All the 5 cases of Nora disease were treated with limited mass. The X-ray and CT showed an under-uniform patchy high-density shadow with a clear boundary. The lesions were not continuous with the intercostal bone marrow cavity. Three lesions Department there is a low density gap. One case of the lesion surface showed a “long-T2” signal on MRI. Microscopically, five lesions showed disorderly arrangement of three components of mature cartilage, bone and fibrous tissue. The cartilage formed a cap-like structure and was doped in the other two components. Some cartilage cells showed mild atypia, Blue dye is characterized by “blue bone”. Conclusion The imaging diagnostic criteria that contribute to Nora disease are as follows: (1) irregular periosteal nodules; (2) lesion diameter more than 30 mm; (3) lesions on X-ray and CT are under-uniform high-density nodules ; (4) low density interstitial space between X-ray and CT; (5) T2-like signal of “cartilage cap” on MRI surface. The final diagnosis still need histopathological examination.