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原发性眼眶结外自然杀伤/T细胞淋巴瘤,鼻型,无鼻受累

Primary Orbital Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type, without Nasal Involvement.

作者信息

Jing Dalan, You Debo, Liu Ziyuan, Wang Wei

机构信息

Department of Ophthalmology, Peking University Third Hospital, Beijing 100191, China.

Beijing Key Laboratory of Restoration of Damaged Ocular Nerve, Peking University Third Hospital, Beijing 100191, China.

出版信息

J Clin Med. 2022 Nov 27;11(23):7010. doi: 10.3390/jcm11237010.

Abstract

Extranodal natural killer/T-cell lymphoma (ENKL) usually occurs in the nose or the nasopharynx, but extranasal and disseminated disease can occur. In this disease, orbital involvement is more commonly seen, but without nasal involvement is rare. A 61-year-old woman was referred with a one-month history of a remarkably enlarging salmon-colored mass arising in the right inner canthus, with redness and painlessness. The motility of the right eye was limited in the medial direction, with external deviation of the eyeball. A magnetic resonance imaging (MRI) scan of the orbits showed a mass of irregular shape located in the right inner canthus, without any sinus involvement. A histopathological examination concluded a diagnosis of primary orbital extranodal natural killer/T-cell lymphoma, nasal type. Her orbital mass significantly reduced to near disappeared after chemotherapy. From the first visit to the present, the survival duration of this patient was more than 1 year. This patient was still alive with a high quality of life and with no systemic metastasis. Extranodal natural killer/T-cell lymphoma, nasal type may primarily arise in the orbit without nasal involvement. Early discovery, early biopsy and diagnosis and early appropriate treatment can successfully control tumors and improve prognosis.

摘要

结外自然杀伤/T细胞淋巴瘤(ENKL)通常发生于鼻腔或鼻咽部,但也可出现鼻外及播散性病变。在这种疾病中,眼眶受累较为常见,但无鼻腔受累的情况罕见。一名61岁女性因右内眦出现一个明显增大的鲑鱼色肿块、伴有发红且无痛,病程1个月前来就诊。右眼内收运动受限,眼球向外偏斜。眼眶磁共振成像(MRI)扫描显示右内眦有一不规则形肿块,无鼻窦受累。组织病理学检查确诊为原发性眼眶结外自然杀伤/T细胞淋巴瘤,鼻型。化疗后她的眼眶肿块明显缩小至几乎消失。从初诊至今,该患者的生存时长已超过1年。该患者仍存活,生活质量高,且无全身转移。鼻型结外自然杀伤/T细胞淋巴瘤可原发于眼眶而无鼻腔受累。早期发现、早期活检及诊断以及早期恰当治疗能够成功控制肿瘤并改善预后。

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