骨外粘液样软骨肉瘤的病理与影像学特点

Pathology and imaging features in extraskeletal myxoid chondrosarcoma

目的探讨骨外粘液样软骨肉瘤的临床、病理与影像学特点,以提高对该病的认识。方法回顾性分析5例经病理证实的骨外粘液样软骨肉瘤患者的临床、影像及病理资料。结果5例患者中男性3例,女性2例,发病年龄为39~73岁。5例患者共包含7个病灶,其中3例单发,2例多发。X线平片(n=3):病灶均呈肿块密度影,其中1例可见斑片状高密度影。CT表现(n=2):病灶呈边界清晰的分叶状低密度肿块,其中1例病灶内见斑点状、结节状钙化影;2例增强扫描显示病灶均有不同程度强化。MRI信号(n=4)表现为等、稍长T1及不均匀长T2信号影,其内见条状分隔;增强扫描1例呈边缘明显强化,3例呈小叶状、结节状明显强化,其内分隔明显强化。大体病理多为分叶状,光镜下可见粘液样间质及条索状纤维组织内瘤细胞浸润。结论骨外粘液样软骨肉瘤发病率极低,影像学表现与组织病理学结构相对应,具有一定诊断意义。

Objective To investigate the clinical,pathological and imaging features of extraskeletal myxoid chondrosarcoma in order to improve the cognition of the disease.Methods The clinical data,pathology and imaging findings of 5 cases with pathologically approved extraskeletal myxoid chondrosarcoma were retrospectively analyzed.Results There were 5 cases,including 3 males and 2 females,with the age range from 39 to 73 years old.7 lesions were found in these cases,including 3 single lesions and 2 multiple lesions.X-ray images(n=3)showed that soft tissue mass was seen in all the lesions,of which 1 case showed patchy high-density.CT(n=2)showed the lesions were low-density masses with clear boundaries.Spotted and nodular calcification shadows were observed in 1 case.The enhanced scan showed different degree of enhancement in both cases.MRI examination(n=4)showed hypo-and iso-intense on T1WI and heterogeneous hyper-intense on T2WI,that contains strip-like septums.On enhanced scanning,1 case showed obvious marginal enhancement,3 cases showed lobular and nodular enhancement,and the internal septums was obviously enhanced.General pathology showed that masses were mostly lobular while myxoid stroma and oncocyte among strip-like fibrous tissue were seen in microscope pathology.Conclusion The incidence of extraskeletal myxoid chondrosarcoma is extremely low,and the imaging findings are corresponding to the histomathological structure,which has certain diagnostic significance.