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眼眶巨大细胞血管纤维瘤,一种孤立性纤维性肿瘤的变异型,发生于一名 16 岁女孩。

Giant cell angiofibroma, a variant of solitary fibrous tumor, of the orbit in a 16-year-old girl.

机构信息

Oncology Service and daggerPathology Department, Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania 19107, USA.

出版信息

Ophthalmic Plast Reconstr Surg. 2009 Sep-Oct;25(5):402-4. doi: 10.1097/IOP.0b013e3181b39a15.

DOI:10.1097/IOP.0b013e3181b39a15
PMID:19966660
Abstract

A 16-year-old girl presented with diplopia and gradual-onset, painless proptosis of the left eye. Orbital CT showed a well-circumscribed, enhancing, extraconal mass in the superior orbit, and the surgical excision was performed. Histopathology was interpreted as capillary hemangioma. Five years later, her symptoms recurred, and she was referred to the Oncology Service, Wills Eye Institute. Repeat orbital MRI showed a well-defined, extraconal mass with loculated areas of enhancement in the left orbit superonasally. Complete surgical excision was performed. Histopathologic examination showed benign, patternless spindle-cell proliferation with prominent intrinsic vascularity and multinucleated giant cells, consistent with giant cell angiofibroma, a variant of solitary fibrous tumor. There was intense immunoreactivity for CD34. After 20 months follow-up, there was no recurrence or development of metastasis. Giant cell angiofibroma, a variant of solitary fibrous tumor, is a rare orbital tumor that presents as a well-circumscribed, enhancing mass and can be found in children.

摘要

一位 16 岁女孩因复视和左眼进行性无痛性眼球突出就诊。眼眶 CT 显示眼眶上方边界清楚、增强的眶外肿块,行手术切除。组织病理学诊断为毛细血管血管瘤。5 年后,她的症状再次出现,转至威尔斯眼研究所肿瘤科。重复眼眶 MRI 显示左眼眶上方呈界限清楚的眶外肿块,伴有分隔区增强。行完全手术切除。组织病理学检查显示良性、无模式梭形细胞增殖,伴有明显的内在血管和多核巨细胞,符合伴有显著血管化的骨巨细胞瘤,是孤立性纤维瘤的一种变体。CD34 免疫反应强烈。20 个月的随访期内,无复发或转移。伴有显著血管化的骨巨细胞瘤,是孤立性纤维瘤的一种变体,是一种罕见的眼眶肿瘤,表现为边界清楚、增强的肿块,可发生于儿童。

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