Prevalence and nature of systemic involvement and stage at initial examination in patients with orbital and ocular adnexal lymphoma

Objective: To determine the stage at initial examination and the prevalence of systemic involvement in patients with orbital and ocular adnexal lymphoma. Methods: The medical records of all patients with orbital and ocular adnexal lymphoma treated in a recent 7-year period were reviewed for stage at initial examination, highest stage during the follow-up period, and recurrence-free survival. Results: Forty-three patients were included. Nineteen patients had mucosa-associated lymphoid tissue, 9 had follicular, 9 had diffuse large-cell, 3 had mantle cell, 2 had small lymphocytic, and 1 had large T-cell lymphoma. The staging workup included chest radiography; orbital computed tomography or magnetic resonance imaging; computed tomography of the chest, abdomen, and pelvis; and bone marrow biopsy. Thirty-six patients had total body positron emission tomography, 7 had gallium scans, and 16 had gastrointestinal endoscopy. Lymphoma stage at diagnosis was IE in 18 patients, II in 6, and IV in 19. Six of 19 patients with mucosa-associated lymphoid tissue, 7 of 9 patients with follicular, 6 of 9 patients with diffuse large-cell, and 3 of 3 patients with mantle cell lymphoma had non-stage IE disease at initial examination. The 5-year recurrence-free survival was 64.6% for the entire cohort. Conclusions: Extraorbital involvement is present at diagnosis in more than half of patients with orbital and ocular adnexal lymphoma and warrants extensive systemic workup at diagnosis, continued surveillance, and consideration of systemic therapy.