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眼眶去分化脂肪肉瘤的罕见诊断。

A rare diagnosis of dedifferentiated liposarcoma of the orbit.

作者信息

Saeed Muhammad Usman, Chang Bernard Y P, Atherley Carolyn, Khandwala Mona, Merchant D W, Liddington Mark

机构信息

Department of Ophthalmology, Clarendon Wing, Leeds General Infirmary, Leeds, UK.

出版信息

Orbit. 2007 Mar;26(1):43-5. doi: 10.1080/01676830600666102.

Abstract

We report the case of a 56-year-old female who presented to the ophthalmic casualty department with proptosis and diplopia. Initially, non-specific orbital inflammation was suspected while investigations were ongoing. A trial of steroids failed to resolve the proptosis to a significant extent. A CT scan showed a well-defined orbital mass, which on orbital biopsy was shown to be a primary orbital liposarcoma of the dedifferentiated variety. This was confirmed by expert histopathological analysis. An exenteration followed by removal of orbital bone was required to achieve total tumour removal. Radiotherapy was delivered to reduce the chance of micro-metastasis. Orbital liposarcoma is an extremely rare tumour. The dedifferentiated variety is even rarer, with only a few reported cases. Lack of information about this potentially aggressive tumour may create therapeutic dilemmas as to the best treatment approach. Similarly, prediction of an accurate prognosis for the patient may be difficult due to the rarity of this condition.

摘要

我们报告了一例56岁女性患者,她因眼球突出和复视而前往眼科急诊室就诊。最初,在进行检查时怀疑为非特异性眼眶炎症。使用类固醇进行的试验未能在很大程度上缓解眼球突出。CT扫描显示有一个边界清晰的眼眶肿物,眼眶活检显示为去分化型原发性眼眶脂肪肉瘤。这通过专家组织病理学分析得到证实。为了实现肿瘤的完全切除,需要进行眼眶内容剜除术并切除眼眶骨。进行了放射治疗以降低微转移的可能性。眼眶脂肪肉瘤是一种极其罕见的肿瘤。去分化型更为罕见,仅有少数病例报道。由于缺乏关于这种潜在侵袭性肿瘤的信息,可能会在最佳治疗方法上产生治疗困境。同样,由于这种疾病的罕见性,可能难以准确预测患者的预后。

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