Pathology and imaging features in extraskeletal myxoid chondrosarcoma
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摘要:
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。 Abstract: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. -
图 1 患者前下腹壁EMC的影像学表现
A：位于下腹部者表现为盆腔左侧类圆形肿块，病灶内可见斑片状稍高密度影（黑箭头所示）；B：病灶呈菜花状软组织肿块，其内可见斑点、斑片状钙化影；C：增强扫描病灶呈强化不均匀；D：MRI示病灶于TIWI呈等信号；E：T2WI呈高信号；F：增强扫描病灶呈明显不均匀强化.
Figure 1. Imaging of extraskeletal myxoid chondrosarcoma of the anterior inferior abdominal wall.
图 2 患者右侧大腿中段占位性病变（黑箭头）
A：DR示病灶与骨骼分界清晰，骨皮质光滑，骨质未见明确受累；MRI示病灶呈稍长T1（B）、长T2（C），内见低信号分隔；D：增强扫描病灶呈不均匀分叶状强化.
Figure 2. Mass in the right mid-thingh of the patient (black arrow).
图 3 右大腿中段软组织占位，TIWI呈稍低信号，T2WI呈混杂高信号，增强扫描呈明显不均匀强化
Figure 3. The tissue lesion in the right-mid-thigh with slightly low signal on T1WI and mixed high signal on T2WI, as well as obvious uneven enhancement on enhanced scan.
表 1 5例EMC患者的临床症状及体征
Table 1. Clinical symptoms and signs of the 5 cases extraskeletal myxoid chondrosarcoma
病例编号 临床症状及体征 检杳方式 预后 病例1 女性，44岁，1年前无明显诱因出现下腹部一“枣子”大小质硬肿物，有按压痛，近期自觉肿物进行性增大，并伴有左下肢及左腹股沟区酸疼就诊。 DR+ CT+MRI 术后3年复发，再次术后3年死亡 病例2 男性，73岁，2年前右大腿因外伤发现大腿肿物未予特殊治疗，大小约15 cmx7 cm，无触痛，无波动。 MRI 无复发，存活 病例3 男性，39岁，6年前发现右大腿中下段一“鸽蛋”大小肿物，无压痛、溃疡、肢体活动受限，现肿物长至“鸡蛋”大小就诊。 DR+MRI 无复发，存活 病例4 男性，39岁，2月前无明显诱因发现右臀部一芒果大小肿物，缓慢增大，质软、活动差，与周围组织粘连，皮肤无异常。 CT+MRI 术后6年复发，再次术后存活 病例5 女性，55岁，1月前自己触及左侧大腿根部软组织两个质硬肿物而就诊，无压痛、溃疡、肢体活动受限。 DR 失访 
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