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附着于视神经的眼眶间叶性软骨肉瘤

Mesenchymal Chondrosarcoma of the Orbit Attached to the Optic Nerve.

作者信息

Bagheri Abbas, Abbaszadeh Mohammad, Torbati Peyman, Rezaei Kanavi Mozhgan

机构信息

Ocular Tissue Engineering Research Center, Shahid Beheshti University of Medical Sciences.

Ophthalmic Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

出版信息

J Craniofac Surg. 2018 Sep;29(6):e591-e594. doi: 10.1097/SCS.0000000000004635.

DOI:10.1097/SCS.0000000000004635
PMID:29863559
Abstract

Mesenchymal chondrosarcoma (MCS) is a rare tumor in the orbit. Although optic nerve displacement is a common finding in intraorbital MCS, optic nerve tissue involvement in tumor has rarely been reported in huge tumors associated with intracranial extension. Herein the authors report a patient with MCS involving optic nerve tissue without intracranial extension. A 59-year-old woman with a 2-month history of progressive proptosis and normal vision presented to us. Computed tomography revealed a clearly outlined heterogeneous mass with calcified foci in its center, which was attached to the optic nerve, magnetic resonance imaging showed the mass to be isointense to gray matter on T1- and T2-weighted images. She underwent lateral orbitotomy and partial tumor excision. Histopathologic study confirmed MCS. She refused exenteration till 1 year but the tumor recurred and her vision decreased to no light perception. Then exenteration was performed with obtaining free margin and she is now free of tumor after 6 months without radiotherapy or chemotherapy. Mesenchymal chondrosarcoma must be differentiated from more common calcified tumors attached to optic nerve like meningioma.

摘要

间叶性软骨肉瘤(MCS)是眼眶内一种罕见的肿瘤。虽然视神经移位是眼眶内MCS的常见表现,但在伴有颅内扩展的巨大肿瘤中,肿瘤累及视神经组织的情况鲜有报道。在此,作者报告1例未发生颅内扩展但累及视神经组织的MCS患者。1名59岁女性因进行性眼球突出2个月且视力正常前来就诊。计算机断层扫描显示一个轮廓清晰的异质性肿块,中心有钙化灶,该肿块附着于视神经;磁共振成像显示该肿块在T1加权像和T2加权像上与灰质等信号。她接受了外侧眼眶切开术和部分肿瘤切除术。组织病理学检查确诊为MCS。她拒绝眶内容摘除术达1年,但肿瘤复发且视力降至无光感。随后进行了眶内容摘除术并获得切缘阴性,目前她在未接受放疗或化疗的情况下,6个月后未发现肿瘤复发。间叶性软骨肉瘤必须与更常见的附着于视神经的钙化性肿瘤如脑膜瘤相鉴别。

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