Department of Pathology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark.
Department of Pathology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark; Department of Ophthalmology, Rigshospitalet-Glostrup, University of Copenhagen, Copenhagen, Denmark.
Surv Ophthalmol. 2019 Jan-Feb;64(1):45-66. doi: 10.1016/j.survophthal.2018.08.002. Epub 2018 Aug 23.
Orbital lymphomas constitute 50-60% of ocular adnexal lymphomas. A total of 2211 cases of orbital lymphoma with a known subtype have been reported in the last 24 years (1994-2017). The vast majority of orbital lymphomas are of B-cell origin (97%), of which extranodal marginal zone B-cell lymphoma (EMZL) (59%) is the most common subtype, followed by diffuse large B-cell lymphoma (23%), follicular lymphoma (9%), and mantle cell lymphoma (5%). Orbital lymphoma is primarily a disease of the elderly. Gender distribution varies according to lymphoma subtype. However, extranodal marginal zone B-cell lymphoma (53%) and follicular lymphoma (75%) show a female predominance, whereas diffuse large B-cell lymphoma shows an even gender distribution. Mantle cell lymphoma has a striking male predominance of 80%. The histopathological subtype and the clinical stage of the disease are the best indicators of prognosis and patient outcome. Low-grade lymphomas such as extranodal marginal zone B-cell lymphoma and FL have a good prognosis, whereas high-grade lymphomas (diffuse large B-cell lymphoma and mantle cell lymphoma) are associated with a poor prognosis. When managing solitary low-grade lymphomas, radiotherapy is the treatment of choice. Chemotherapy, with or without radiotherapy, should be chosen for disseminated and high-grade lymphomas.
眼眶淋巴瘤占眼附属器淋巴瘤的 50-60%。在过去的 24 年（1994-2017 年）中，已经报道了 2211 例已知亚型的眼眶淋巴瘤病例。绝大多数眼眶淋巴瘤来源于 B 细胞（97%），其中结外边缘区 B 细胞淋巴瘤（EMZL）（59%）是最常见的亚型，其次是弥漫性大 B 细胞淋巴瘤（23%）、滤泡性淋巴瘤（9%）和套细胞淋巴瘤（5%）。眼眶淋巴瘤主要发生于老年人。性别分布根据淋巴瘤亚型而异。然而，结外边缘区 B 细胞淋巴瘤（53%）和滤泡性淋巴瘤（75%）以女性为主，而弥漫性大 B 细胞淋巴瘤则呈性别分布均匀。套细胞淋巴瘤以 80%的显著男性优势为主。组织病理学亚型和疾病临床分期是预后和患者结局的最佳指标。低级别淋巴瘤如结外边缘区 B 细胞淋巴瘤和 FL 预后良好，而高级别淋巴瘤（弥漫性大 B 细胞淋巴瘤和套细胞淋巴瘤）则预后较差。在管理孤立性低级别淋巴瘤时，放射治疗是首选治疗方法。对于播散性和高级别淋巴瘤，应选择化疗联合或不联合放疗。
