Du Baixue, He Xin, Wang Yujiao, He Weimin
Department of Ophthalmology, West China Hospital of Sichuan University, No. 37 Guoxue Xiang, Wuhou District, Chengdu, 610041, Sichuan Province, China.
Department of pathology, West China Hospital of Sichuan University, No. 37 Guoxue Xiang, Wuhou District, Chengdu, 610041, Sichuan Province, China.
BMC Ophthalmol. 2020 Jul 6;20(1):264. doi: 10.1186/s12886-020-01458-1.
Although myxofibrosarcoma (MFS) is the most common mesenchymal tumor, occurrence in the orbit is extremely rare. A characteristic clinical feature of MFS is its propensity for local recurrence (LR). Still, none of published literature has described the entire clinical course of multiple recurrences of MFS in the orbit. Here we present an unusual case in which a patient with multiple recurrences of MFS in the orbit followed-up for 5 years. We describe its clinical-pathological correlation, treatment, and prognosis.
A 70-year-old woman sought treatment for a year history of right upper eyelid swelling in May 2014. Since then, she underwent three surgical procedures in the right orbit in the same region (in 2014, 2016, and 2017). The pathology analysis of the three surgical tissue samples was consistent with myxofibrosarcoma, characterized by spindle-shaped cells and variably myxoid stroma. In the 2 years follow-up after the third surgery, there was no evidence of tumor recurrence.
Myxofibrosarcoma of the orbit is extremely rare. Since few reports are available in the literature, the diagnosis and management of the orbit myxofibrosarcoma is still a challenge to ophthalmologists. Histopathology is highly valuable in the diagnosis. As a clinical characteristic, the lesions have a high risk of local recurrence; therefore, aggressive resection and careful postoperative follow-up are paramount.
尽管黏液纤维肉瘤（MFS）是最常见的间叶组织肿瘤，但在眼眶中发生极为罕见。MFS的一个典型临床特征是其局部复发（LR）倾向。然而，已发表的文献均未描述眼眶MFS多次复发的完整临床过程。在此，我们报告一例不寻常的病例，一名眼眶MFS多次复发的患者接受了5年的随访。我们描述了其临床病理相关性、治疗及预后情况。
一名70岁女性于2014年5月因右上眼睑肿胀1年病史前来就诊。自那时起，她在右眼眶同一区域接受了三次手术（分别在2014年、2016年和2017年）。三次手术组织样本的病理分析均符合黏液纤维肉瘤，其特征为梭形细胞和不同程度的黏液样间质。在第三次手术后的2年随访中，未发现肿瘤复发迹象。
眼眶黏液纤维肉瘤极为罕见。由于文献报道较少，眼眶黏液纤维肉瘤的诊断和治疗对眼科医生来说仍是一项挑战。组织病理学在诊断中具有很高的价值。作为一种临床特征，病变具有较高的局部复发风险；因此，积极切除和仔细的术后随访至关重要。
