Park Sung Chul, Oh Dong Eun, Suh Yeon-Lim, Kim Yoon-Duck
Department of Ophthalmology, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Ilwon-dong, Kangnam-ku, Seoul, 135-710, Korea.
Ophthalmic Plast Reconstr Surg. 2007 May-Jun;23(3):246-8. doi: 10.1097/IOP.0b013e31803eb1b9.
A 34-year-old woman presented with progressive proptosis of the right eye for 2 years. MRI revealed a well-demarcated mass in the right intraconal space, which was isointense on T(1)- and hyperintense on T(2)-weighted images with bright uniform enhancement. The mass was resected through a lateral orbitotomy. Histopathologic examination revealed cords and clusters of spindle and epithelioid cells embedded in a myxoid matrix, with vacuolated eosinophilic cytoplasm and round nuclei. The tumor cells were diffusely positive for epithelial membrane antigen and vimentin. These findings helped confirm the diagnosis of chordoid meningioma, a rare variant of meningioma with a greater risk of recurrence and aggressive growth. Many of the clinical manifestations and imaging findings were indistinguishable from those of cavernous hemangiomas, solitary fibrous tumors, neurofibromas, and other variants of meningiomas. Given the prognostic significance of chordoid meningioma, extra care and attention should be given during surgery, histopathologic assessment, and follow-up.
一名34岁女性因右眼进行性眼球突出2年就诊。磁共振成像（MRI）显示右侧肌锥内有一个边界清晰的肿块，在T1加权像上呈等信号，在T2加权像上呈高信号，并有均匀明亮强化。通过外侧眶切开术切除该肿块。组织病理学检查显示，梭形细胞和上皮样细胞呈条索状和团块状，包埋于黏液样基质中，胞质呈空泡状嗜酸性，细胞核圆形。肿瘤细胞上皮膜抗原和波形蛋白弥漫性阳性。这些发现有助于确诊脊索样脑膜瘤，这是一种罕见的脑膜瘤变异型，复发风险更高，生长更具侵袭性。其许多临床表现和影像学表现与海绵状血管瘤、孤立性纤维瘤、神经纤维瘤及其他脑膜瘤变异型难以区分。鉴于脊索样脑膜瘤的预后意义，手术、组织病理学评估及随访过程中应格外小心并予以关注。
