Department of Ophthalmology, Faculty of Medicine, School of Health Sciences, University of Ioannina, Ioannina, Greece.
Department of Pathology, Faculty of Medicine, School of Health Sciences, University of Ioannina, Ioannina, Greece.
Clin Exp Med. 2018 May;18(2):151-163. doi: 10.1007/s10238-017-0474-1. Epub 2017 Sep 22.
Ocular adnexal lymphomas are a group of heterogeneous neoplasms representing approximately 1-2% of non-Hodgkin lymphomas and 8% of extranodal lymphomas. The incidence of primary ocular adnexal lymphoid tumors has raised over the last decades, and this could be probably attributed to the more sophisticated diagnostic techniques. Due to the wide spectrum of clinical manifestations, ocular tissue biopsy is important in order to set a precise diagnosis based on histological, immunophenotypical and, in some cases, molecular findings. The most common subtype, which may account for up to 80% of primary ocular adnexal lymphomas, is extranodal marginal zone lymphoma (EMZL) of mucosa-associated lymphoid tissue. This lymphoma is usually asymptomatic in the early phase of the disease causing a delay in the final diagnosis and prompt therapy. The pathogenesis of a proportion of these tumors has been linked to chronic inflammatory stimulation from specific infectious factors (e.g., Chlamydia psittaci) or to autoimmunity. The further improvement in diagnostic methods and the further understanding of the pathogenesis of ocular adnexal EMZL may contribute to the establishment of a more successful multidisciplinary therapeutic planning.
眼附属器淋巴瘤是一组异质性肿瘤，约占非霍奇金淋巴瘤的 1-2%，占结外淋巴瘤的 8%。过去几十年来，原发性眼附属器淋巴组织肿瘤的发病率有所上升，这可能归因于更复杂的诊断技术。由于临床表现广泛，眼组织活检对于根据组织学、免疫表型和在某些情况下的分子发现进行精确诊断非常重要。最常见的亚型是黏膜相关淋巴组织的结外边缘区淋巴瘤 (EMZL)，可能占原发性眼附属器淋巴瘤的 80%。这种淋巴瘤在疾病的早期通常无症状，导致最终诊断和及时治疗的延迟。这些肿瘤的一部分发病机制与特定感染因素（如鹦鹉热衣原体）引起的慢性炎症刺激或自身免疫有关。进一步提高诊断方法并进一步了解眼附属器 EMZL 的发病机制，可能有助于制定更成功的多学科治疗计划。
