眼眶间叶性软骨肉瘤--病例报告与文献复习

Mesenchymal chondrosarcoma of the orbit:report of a case and review of the literature

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。

AIM： To report a rare case of mesenchymal chondrosarcoma in the orbit and to explore its clinic manifestations, pathologic characters, management and prognosis. METHODS： We report a case of rnesenchyrnal chondrosarcorna of the orbit. The clinical materials, including ophthalmological examination, computed tornography scan of the orbit, histopathology and irnrnunohistochernistry of the biopsy specimen was reported, and its pertinent literatures were reviewed. RESULTS： A 36-year-old female was seen with proptosis and decreased vision. Histopathology demonstrated an admixture of undifferentiated rnesenchyrnal cells and islands of mature hyaline cartilage. Irnrnunohistochernical studies revealed positivity for virnentin and S-100, which was consistent with the diagnosis of rnesenchyrnal chondrosarcorna. CONCLUSION： Mesenchyrnal chondrosarcorna in the orbit is extremely rare malignant tumor. Multi-rnodality treatments （surgery, chemotherapy and radiotherapy） may lead to long-term survival.