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眼眶自然杀伤/T 细胞淋巴瘤:综合病例系列及文献综述

英文原题:Orbital natural killer/T-cell lymphoma: a comprehensive case series and literature review.

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Orbital natural killer/T-cell lymphoma: a comprehensive case series and literature review.

PubMed 2025/02/28(内容时间) BMC Cancer Q2 · IF 4.1(JCR 2025)

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研究概要

眼眶 NKTCL 具有高度侵袭性、进展迅速、预后差。其临床症状与眶蜂窝织炎或炎性假瘤相似。当抗炎和皮质类固醇治疗无效时,强烈建议早期活检以进行组织病理学确诊。一旦确诊,应及时启动化疗和放疗,以改善患者预后。

研究思路结论见上方概要

评估眼眶自然杀伤/T细胞淋巴瘤(NKTCL)患者的临床特征、病理表现、治疗及预后。

本回顾性研究收集了2005年11月至2024年3月期间在首都医科大学北京同仁医院经病理检查诊断为眼眶NKTCL的11例患者的临床资料。对患者进行随访以评估其预后。

眼眶NKTCL患者的中位年龄为51岁,男性略占优势。所有病例均为单侧,临床表现为眼睑或眶周肿胀,其他症状包括视力下降(90.9%)、结膜充血(90.9%)、眼球运动障碍(81.8%)、眼球突出(63.6%)和上睑下垂(36.4%)。所有患者均有周围组织受累,最常见的部位为眼睑(11例)、眼球壁(7例)和泪腺(5例)。此外,63.6%的患者合并鼻部软组织受累,而36.4%为孤立性眼眶NKTCL。病理检查显示非典型淋巴细胞弥漫性增生和浸润,偶伴坏死。免疫组化染色显示GrB(11例)、CD3(10例)、TIA1(9例)、CD56(8例)和EBV/EBER(10例)阳性表达。所有患者均接受眼部手术,7例接受术后化疗,1例接受同期局部放疗。随访(中位时间:120个月)显示预后较差,5例失访,5例死亡,仅1例无复发生存。

展开英文摘要原文

To evaluate the clinical features, pathological findings, treatment, and prognosis of patients with orbital natural killer/T-cell lymphoma (NKTCL).

This retrospective study collected clinical data from 11 patients diagnosed with orbital NKTCL by pathological examination at Beijing Tongren Hospital, Capital Medical University, between November 2005 and March 2024. The patients were followed up to assess their prognosis.

The median age of patients with orbital NKTCL was 51 years, with a slight male predominance. All cases were unilateral, with clinical presentations of eyelid or periorbital swelling, and other symptoms including decreased vision (90.9%), conjunctival congestion (90.9%), ocular motility disorders (81.8%), proptosis (63.6%), and ptosis (36.4%). All patients had surrounding tissue involvement, with the most common sites being the eyelid (11 cases), eyeball wall (7 cases), and lacrimal gland (5 cases). Additionally, 63.6% of patients had concurrent soft tissue involvement of the nasal region, while 36.4% had isolated orbital NKTCL. Pathological examination revealed diffuse proliferation and infiltration of atypical lymphocytes, occasionally with necrosis. Immunohistochemical staining showed positive expression of GrB (11 cases), CD3 (10 cases), TIA1 (9 cases), CD56 (8 cases), and EBV/EBER (10 cases). All patients underwent ocular surgery, 7 received postoperative chemotherapy, and one received concurrent local radiotherapy. Follow-up (median duration: 120 months) revealed poor prognosis, with 5 patients lost to follow-up, 5 deaths, and only one survival without recurrence.

Orbital NKTCL is highly aggressive, rapidly progressive, and has a poor prognosis. Its clinical symptoms are similar to orbital cellulitis or inflammatory pseudotumor. When anti-inflammatory and corticosteroid therapies prove ineffective, early biopsy for histopathological confirmation is strongly recommended. Upon diagnosis, prompt chemotherapy and radiotherapy should be initiated to improve the patient's prognosis.

论文信息

作者
Li J、Ren T、Liu R、Zhang H、Wang N、Guo Q、Xu L、Ma J
第一作者单位
Beijing Institute of Ophthalmology, Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, 100730, China.China
通讯作者单位
Beijing Institute of Ophthalmology, Beijing Tongren Eye Center, Beijing Tongren Hospital, Capital Medical University, Beijing, 100730, China. jmma@sina.com.China
文献类型
病例报告 · 综述
期刊
BMC cancer2025 Feb 28
原文标识
PubMed 40022004 · DOI 10.1186/s12885-025-13761-5