Ocular adnexal lymphoma: clinical behavior of distinct World Health Organization classification subtypes

• Published in: International journal of radiation oncology, biology, physics Vol. 57; no. 5; p. 1382
• Main Authors: Fung, Claire Y, Tarbell, Nancy J, Lucarelli, Mark J, Goldberg, Saveli I, Linggood, Rita M, Harris, Nancy L, Ferry, Judith A
• Format: Journal Article
• Language: English
• Published: United States 01-12-2003
• Subjects: Adolescent; Adult; Aged; Aged, 80 and over; Child; Dose-Response Relationship, Radiation; Eye Diseases - etiology; Eye Neoplasms - mortality; Eye Neoplasms - pathology; Eye Neoplasms - radiotherapy; Female; Humans; Lymphoma, B-Cell, Marginal Zone - mortality; Lymphoma, B-Cell, Marginal Zone - pathology; Lymphoma, B-Cell, Marginal Zone - radiotherapy; Lymphoma, Follicular - mortality; Lymphoma, Follicular - pathology; Lymphoma, Follicular - radiotherapy; Lymphoma, Non-Hodgkin - mortality; Lymphoma, Non-Hodgkin - pathology; Lymphoma, Non-Hodgkin - radiotherapy; Male; Middle Aged; Orbital Neoplasms - radiotherapy; Orbital Neoplasms - secondary; Radiotherapy - adverse effects; Radiotherapy Dosage; Recurrence; Survival Rate; Treatment Outcome
• ISSN: 0360-3016
• DOI: 10.1016/S0360-3016(03)00767-3

To evaluate the clinical behavior and treatment outcome of ocular adnexal lymphomas classified by the World Health Organization system, with emphasis on marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT).
The clinicopathologic materials from 98 consecutive patients treated for ocular adnexal lymphoma were reviewed. Fourteen patients had prior lymphoma and 84 patients had primary disease (75% Stage I, 6% Stage III, and 19% Stage IV). Radiation (photons/electrons) was administered to 102 eyes to a median dose of 30.6 Gy. The mean follow-up was 82 months.
The most common subtypes among the primary patients were MALT (57%) and follicular (18%) lymphoma. The 5-year actuarial local control rate in 102 irradiated eyes was 98%. Among the low-grade lymphomas, the 5-year local control rate correlated with the radiation dose in the MALT lymphoma subgroup (n = 53): 81% for <30 Gy and 100% for > or =30 Gy (p <0.01). For the non-MALT low-grade lymphomas such as follicular lymphoma (n = 30), the local control rate was 100% regardless of dose. For 39 Stage I MALT lymphoma patients treated with radiation alone, the distant relapse-free survival rate was 75% at 5 years and 45% at 10 years. Distant relapses were generally isolated and successfully salvaged by local therapy. The overall survival for this subgroup was 81% at 10 years, with no deaths from lymphoma.
Dose-response data suggest that the optimal radiation dose for MALT lymphoma of the ocular adnexa is 30.6-32.4 Gy in 1.8-Gy fractions and follicular lymphoma is adequately controlled with doses in the mid-20 Gy range. The substantial risk of distant relapse in Stage I ocular adnexal MALT lymphoma underscores the importance of long-term follow-up for this disease and the need for additional comparative studies of MALT lymphoma of different anatomic sites.