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Elham Hatef, MD; Dianna Roberts, PhD; Peter McLaughlin, MD; Barbara Pro, MD; Bita Esmaeli, MD

Arch Ophthalmol. 2007;125(12):1663-1667.

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.

Author Affiliations: Section of Ophthalmology (Drs Hatef and Esmaeli), Department of Head and Neck Surgery (Drs Hatef, Roberts, and Esmaeli), and Department of Lymphoma and Myeloma (Drs McLaughlin and Pro), The University of Texas M. D. Anderson Cancer Center, Houston.

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