Title : Analysis of clinical and pathological features of 187 cases of primary ocular adnexal lymphoma
Abstract:
Objective: To investigate the clinical and pathological features of primary ocular adnexal lymphoma (OAL). Methods A total of 187 OAL cases diagnosed in Xi'an People's Hospital (Xi'an Fourth Hospital) and Shaanxi Provincial Eye Hospital from 2016 to 2024 were enrolled, including 132 cases of extranodal marginal zone B-cell lymphoma (EZML), 21 cases of large B-cell lymphoma(LBCL), 10 cases of follicular lymphoma (FL) , 9 cases of mantle cell lymphoma (MCL), 7 cases of extranodal NK/T cell lymphoma (NKTCL), 6 cases of plasma cell neoplasms (PCN), 1 case of plasmablastic lymphoma and 1 case of B lymphoblastic lymphoma. The clinical manifestations, imaging features and pathological characteristics of each case were collected.
Results: The 187OAL patients were aged 1~89 years, with an average age of 60 years, a median age of 62 years, a male-to-female ratio of 118:69, 87 cases of left eye, 98 cases of right eye, and 2 cases of both eyes, mainly occurring in the orbit, conjunctiva, lacrimal gland and eyelids. The clinical manifestations of patients are mostly found to involve the ocular appendage, and some patients are accompanied by symptoms such as eyelid conjunctival swelling, proptosis, ocular motility disorder or visual field loss, and the imaging manifestations are irregular soft tissue density shadows at the ocular appendage site, which can form obvious nodules and infiltrative growth. In some cases, tumor tissue invaded and destroyed surrounding tissues, including 2 cases of nasal sinuses, 2 cases of maxillary sinuses, and 1 case of surrounding bone tissue.The pathological features were diffuse neoplastic lymphocyte proliferation as the main manifestation, and the cell morphology ranged from mature small lymphocytes to large cells and even anaplastic tumor cells, and all types of lymphoma had the same tissue structure as lymphoma in other sites, accompanied by corresponding molecular changes. All patients underwent mass resection, and some patients were followed by chemoradiotherapy. Conclusion As a rare site of extranodal lymphoma, OAL occurs in the orbit, conjunctiva, lacrimal gland and eyelids, accounting for only 1%~2% of non-Hodgkin lymphoma and 5%~15% of extranodal lymphoma, but it is a common malignant tumor of ocular. As a group of heterogeneous tumors, indolent low-grade lymphoma is more common, and there is no lack of high-grade aggressive lymphoma, with diverse clinical manifestations and pathological tissue morphology, often requiring immunohistochemistry and molecular testing to assist in diagnosis. Tumor tissue invasion and destruction of surrounding tissues in some cases, including 2 cases of nasal sinuses, 2 cases of maxillary sinuses, and 1 case of invasion of surrounding bone tissue, the pathological characteristics were diffuse neoplastic lymphocyte proliferation as the main manifestation, and the cell morphology ranged from mature small lymphocytes to large cells and even anaplastic tumor cells, and all types of lymphoma were the same as those of lymphoma at other sites, with corresponding molecular changes. All patients underwent mass resection, and some patients were followed by chemoradiotherapy. Conclusion As a rare site of extranodal lymphoma, OAL occurs in the orbit, conjunctiva, lacrimal gland and eyelids, accounting for only 1%~2% of non-Hodgkin lymphoma and 5%~15% of extranodal lymphoma, but it is a common malignant tumor of ocular appendage. As a group of heterogeneous tumors, indolent low-grade lymphoma is more common, and there is no lack of high-grade aggressive lymphoma, with diverse clinical manifestations and pathological tissue morphology, often requiring immunohistochemistry and molecular testing to assist in diagnosis.
