Ocular Adnexal Lymphoma of the Lacrimal Drainage Apparatus: Clinical Characteristics, Radiographic Features, and Management

Abstract

PURPOSE: To review and analyze lymphoid tumors involving the lacrimal drainage apparatus (LDA) through retrospective review of an orbital surgeon's (T.J.S.) practice across multiple centers, including incidence, clinical presentation, signs, radiological features, management, and mortality. METHOD: Retrospective cohort analysis of ocular and adnexal lymphoproliferative disease (OALD) seen through Queensland orbital services between 1992 and 2024. Inclusion criteria were patients with histologically proven OALD. RESULTS: Twenty-two patients with OALD demonstrating involvement of the LDA were identified. Fifty percent of patients presented with dacryocystitis, 32% with a palpable mass in the medial canthus and 27% with symptomatic epiphora. However, 7 (30%) patients had disease detected incidentally at the time of elective dacryocystorhinostomy surgery. CT findings included variable patterns of bony remodeling, erosion, or destruction depending on histology and nasolacrimal duct obstruction. MRI, when utilized, better defined the extent of lesions; commonly isointense on T1 and T2-weighted imaging and moderately gadolinium enhancing, with positron emission tomography-CT effective at screening for undiagnosed systemic disease. Histological review highlighted 8 cases (38%) of diffuse large B-cell lymphoma, 4 (18%) T-cell lymphoma, 3 (14%) chronic lymphocytic or small lymphocytic lymphoma, and 7 (30%) other variants. Ann Arbor lymphoma classification highlighted 30% (6) of patients with stage 1, with 60% (13) identified at stage 3 or 4. Ten patients have achieved disease-free survival, with 1 patient requiring ongoing treatment. Three patients died due to OALD. CONCLUSION: OALD affecting the LDA, while rare, represents a potentially life-threatening differential for patients with LDA symptoms. Presentations are variable; early detection, timely imaging, and histological identification can meaningfully affect associated mortality.