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September 2004


Arch Ophthalmol. 2004;122(9):1382. doi:10.1001/archopht.122.9.1382

Ocular Adnexal Lymphoma: Clinical Behavior of Distinct World Health Organization Classification Subtypes

Purpose: To evaluate the clinical behaviorand treatment outcome of ocular adnexal lymphomas classified by the WorldHealth Organization Classification, with emphasis on marginal zone lymphomaof mucosa-associated lymphoid tissue (MALT).

Methods: The clinico-pathologic materials werereviewed in 98 consecutive patients treated for ocular adnexal lymphoma. Fourteenpatients had prior lymphoma while 84 patients had primary disease (75% stageI, 6% stage III, 19% stage IV.) Radiation (photons/electrons) was administeredto 102 eyes to 30.6 Gy median dose. Mean follow-up was 82 months.

Results: The most common subtypes among primarypatients were MALT (57%) and follicular (18%) lymphoma. The 5-year actuariallocal control rate in 102 irradiated eyes was 98%. Among the low-grade lymphomas,5-year local control was correlated with radiation dose in the MALT lymphomasubgroup (N = 53): 81% for <30 Gy and 100% for ≥30Gy (p<0.01). Forthe non-MALT low-grade lymphomas such as follicular lymphoma (N = 30), localcontrol was 100% regardless of dose. For 39 stage I MALT lymphoma patientstreated with radiation alone, the distant-relapse-free survival was 75% at5 years and 45% at 10 years. Distant relapses were generally isolated andsuccessfully salvaged by local therapy. The overall survival for this subgroupwas 81% at 10 years, with no deaths from lymphoma.

Conclusions: Dose-response data suggest theoptimal radiation dose for MALT lymphoma of the ocular adnexa is 30.6-32.4Gy in 1.8 Gy fractions while follicular lymphoma is adequately controlledwith doses in the mid 20 Gy range. The substantial risk of distant relapsein stage I ocular adnexal MALT lymphoma underscores the importance of long-termfollow-up for this disease and the need for additional comparative studiesof MALT lymphoma of different anatomical sites. (2003;57:1382-1391)