Aims To examine whether the specific location of ocular adnexal lymphoma (OAL) and the American Joint Committee on Cancer (AJCC) TNM tumour stage are prognostic factors for mortality in the main OAL subtypes.
Methods Clinical and survival data were retrospectively collected from seven international eye cancer centres. All patients from 1980 to 2017 with histologically verified primary or secondary OAL were included. Cox regression was used to compare the ocular adnexal tumour locations on all-cause mortality and disease-specific mortality.
Results OAL was identified in 1168 patients. The most frequent lymphoma subtypes were extranodal marginal zone B-cell lymphoma (EMZL) (n=688, 59%); follicular lymphoma (FL) (n=150, 13%); diffuse large B-cell lymphoma (DLBCL) (n=131, 11%); and mantle cell lymphoma (MCL) (n=89, 8%). AJCC/TNM tumour-stage (T-stage) was significantly associated with disease-specific mortality in primary ocular adnexal EMZL and increased through T-categories from T1 to T3 disease. No associations between AJCC/TNM T-stage and mortality were found in primary ocular adnexal FL, DLBCL, or MCL. EMZL located in the eyelid had a significantly increased disease-specific mortality compared with orbital and conjunctival EMZL, in both primary EMZL and the full EMZL cohort. In DLBCL, eyelid location had a significantly higher disease-specific mortality compared with an orbital or lacrimal gland location.
Conclusion Disease-specific mortality is associated with AJCC/TNM T-stage in primary ocular adnexal EMZL patients. Lymphoma of the eyelid has the highest disease-specific mortality in primary EMZL, and in the full cohort of EMZL and DLBCL patients.
- eye lids
- lacrimal gland
Data availability statement
Data are available on reasonable request.
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Contributors Study concept and design: SDV, PKR, SEC, BE and SH. Acquisition, analysis or interpretation of data: SDV, SEC, BE, PTF, GG, HEG, TGH, FH, SGH, JJK, MKK, PAM, LHM, KM, PKR, VDS, LDS, MCS, BT, GV and SH. Drafting of manuscript: SDV, SEC, LHM, VDS and SH. Critical revision of the manuscript for important intellectual content: SDV, SEC, BE, PTF, GG, HEG, TGH, FH, SGH, JJK, MKK, PAM, LHM, KM, PKR, VDS, LDS, MCS, BT, GV and SH. Statistical analysis: SDV, VDS. Obtained funding: SH. Administrative, technical or material support: SDV, SEC, BE, PTF, GG, HEG, TGH, FH, SGH, JJK, MKK, PAM, LHM, KM, PKR, VDS, LDS, MCS, BT, GV and SH. Study supervision: FH, LHHM, PKR, VDS and SH.
SDV, SH had full access to the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis.
Funding The Eye Cancer Foundation supported this study with an unrestricted fellowship grant for Gerardo Graue with grant number GG1.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.