Objective: To investigate the clinical manifestation and pathological features of primary ocular adnexal non-Hodgkin lymphoma.
Methods: Data of 18 patients with biopsy-proven ocular adnexal non-Hodgkin lymphoma at Xiangya hospital were reviewed.The disease site, clinical manifestation,imaging and pathological features of the tumor were summarized.
Results: All patients had typical presentation of an adnexal mass.Twelve(66.7%) patients had orbital involvement, 3(16.7%) had conjunctival, and 3(16.7%) had lymphoma involving the eyelids.Eight patients were misdiagnosed as "inflammatory pseudotumour" before the operation according to their clinical and imaging examination,another 8 patients were diagnosed as "ocular adnexal tumour with unknown nature" before the operation. According to the pathologic diagnosis, 16 patients (88.9%)had marginal zone lymphomas of mucosa-associated lymphoid tissue(MZL-MALT) and 2 (11.1%) had NK/T-cell lymphoma.
Conclusion: The typical presentation of ocular adnexal lymphoma is a painless mass.Orbital connective tissue is the most involved anatomical site. The diagnosis of ocular adnexal non-Hodgkin lymphoma is difficult which could easily be misdiagnosed as "inflammatory pseudotumour". MZL-MALT is the most common pathologic type.