· AIM: To report a rare case of mesenchymalchondrosarcoma in the orbit and to explore its clinicmanifestations, pathologic characters, management andprognosis. · METHODS: We report a case of mesenchymalchondrosarcoma of the orbit. The clinical materials,including ophthalmological examination, computed tomo-graphy scan of the orbit, histopathology and immunohis-tochemistry of the biopsy specimen was reported, and itspertinent literatures were reviewed.· RESULTS: A 36-year-old female was seen with proptosisand decreased vision. Histopathology demonstrated anadmixture of undifferentiated mesenchymal cells andislands of mature hyaline cartilage. Immunohistochemicalstudies revealed positivity for vimentin and S-100, whichwas consistent with the diagnosis of mesenchymalchondrosarcoma.· CONCLUSION: Mesenchymal chondrosarcoma in theorbit is extremely rare malignant tumor. Multi-modalitytreatments (surgery, chemotherapy and radiotherapy)may lead to long-term survival.%目的:报道1例罕见的眼眶间叶性软骨肉瘤,探讨其临床表现、病理特点、治疗以及预后.方法:详细报告1例眼眶间叶性软骨肉瘤病例的临床资料,并检索迄今国内所有该病例报道,结合国外文献进行分析总结.结果:该病例非常罕见,迄今国外报道仅18例,国内报道仅8例.本文报告的1例36岁女性患者,2次手术,1次误诊为血管外皮瘤,2a后因复发行2次手术,术后病理检查:肿瘤组织由未分化的间叶瘤细胞及岛状的软骨细胞组成,免疫组织化学检查:Vimentin(++),S-100(+),病理报告为右眼眶间叶性软骨肉瘤.结论:间叶性软骨肉瘤是发生于有成软骨潜能的原始间叶组织的恶性肿瘤,发生于眼眶者罕见,综合治疗(手术、化学治疗以及放射治疗)可减少复发、提高长期生存率.
展开▼
