眼眶间叶性骨外软骨肉瘤。1例报告并文献复习。
Mesenchymal extraskeletal chondrosarcoma of the orbit. Report of a case and review of the literature.
作者信息
Khouja N, Ben Amor S, Jemel H, Kchir N, Boussen H, Khaldi M
机构信息
Service de Neurochirurgie, Institut National de Neurologie, Tunis, Tunisie.
出版信息
Surg Neurol. 1999 Jul;52(1):50-3. doi: 10.1016/s0090-3019(99)00033-6.
BACKGROUND
Extraskeletal mesenchymal chondrosarcoma (MCS) is relatively uncommon. Orbital location is extremely rare: only 16 cases have been reported until now. We report a case of extraskeletal mesenchymal chondrosarcoma in a 27-year-old man and review the literature on its manifestations and management.
CASE REPORT
This patient had a 2-year history of progressive proptosis of the right eye. Skull X-ray and CT scan showed intraorbital calcification and a large lesion in the upper right orbit. He was operated three times because of recurrence of the tumor. The last recurrence was observed to have extension to the intracranial region, detected on MRI and CT scan. This secondary extension of the tumor to the intracranial region has not been previously reported. Immunohistochemical analysis for S-100 protein showed focal positivity.
CONCLUSION
Mesenchymal chondrosarcoma of the orbit is rare, and secondary extension to the intracranial region has not previously been reported.
背景
骨外间叶性软骨肉瘤（MCS）相对少见。眼眶部位极为罕见：迄今为止仅报道过16例。我们报告一例27岁男性的骨外间叶性软骨肉瘤病例，并复习其临床表现及治疗的相关文献。
病例报告
该患者有右眼进行性眼球突出2年病史。头颅X线及CT扫描显示眶内钙化及右上眼眶巨大病变。因肿瘤复发，他接受了3次手术。最后一次复发经MRI及CT扫描发现肿瘤已扩展至颅内区域。此前尚未报道过肿瘤向颅内区域的这种继发性扩展。S-100蛋白免疫组化分析显示局灶性阳性。
结论
眼眶间叶性软骨肉瘤罕见，此前尚未报道过向颅内区域的继发性扩展。
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