| Abstract [eng] |
Introduction. Ocular adnexal lymphoma (OAL) is a rare condition, but one of the most common ocular adnexal tumors. It has diverse clinical manifestations and variable progression depending on the subtype. OAL is primarily categorized as non-Hodgkin's B-cell lymphoma. Due to the increasing prevalence, varied clinical course, and multiple treatment approaches, the disease poses important diagnostic and therapeutic challenges. Aim. This study aims to provide a comprehensive review of primary OAL's epidemiology, etiopathogenesis, clinical features, subtypes, diagnostic methods, and treatment strategies, due to the limited detailed data available in the literature. Tasks. To analyze subtype differences, localization characteristics, and treatment choices based on lymphoma type and prognosis. Methods. Literature sources were selected through a targeted search in PubMed and Google Scholar. Articles discussing OAL, its epidemiology, etiopathogenesis, classification, clinical signs, diagnostics, and treatment were included. Preference was given to peer-reviewed and recent sources. A total of 74 sources were analyzed, 68 published in the last decade. Results. OAL primarily affects older adults, with the prevalence, particularly of extranodal marginal zone lymphoma (EMZL), steadily rising. The etiopathogenesis of OAL is linked to chronic antigenic stimulation caused by specific microorganisms or infections. Chlamydia psittaci is a significant pathogen associated with EMZL. Other contributing factors include viral infections (e.g., EBV, HCV, HIV), autoimmune diseases, and genetic predisposition. Primary OAL generally manifests as a slow-growing, painless tumor that can involve various ocular structures, such as the orbit, lacrimal gland, lacrimal sac, conjunctiva, and eyelids. Common symptoms include globe protrusion, swelling, diplopia, restricted movement, and less frequently, visual disturbances. The clinical presentation depends on tumor localization and characteristics, with aggressive lymphomas causing faster progression, optic nerve damage, or systemic symptoms. Treatment involves radiotherapy, chemotherapy, and immunotherapy, with adjustments required in cases of relapse. Prognosis depends on the lymphoma subtype and stage. Extranodal marginal zone lymphoma has a favorable prognosis, treated with radiotherapy. Diffuse large B-cell lymphoma requires chemotherapy with variable outcomes. Follicular lymphoma responds best to radiotherapy. Mantle cell lymphoma has a poor prognosis, but chemotherapy with rituximab can improve outcomes. Conclusions. OAL is the most common form of orbital tumor, with its clinical course and treatment dependent on lymphoma subtype. The etiopathogenesis is linked to infections, autoimmune diseases, and genetic factors. Treatment and prognosis depend on lymphoma type and stage. Personalized treatment, considering lymphoma aggressiveness and the patient’s condition, is essential for optimal outcomes. |
