Alexandra I. Manta, Alexander Vittorio, Timothy J. Sullivan
Purpose: T-cell lymphomas involving the orbit and ocular adnexa are exceedingly rare if compared to the more common B-cell malignancies, representing 1-3% of orbital lymphomas. The vast majority of these tumours are sec- ondary to peripheral T-cell lymphomas already present elsewhere in the body but reports of primary T-cell malig- nancies affecting the orbital tissues have confirmed its previously doubted existence. Our experience with both primary and secondary cases over a 30-year period may better characterise these aggressive tumours.
Methods: This is a retrospective observational study of patients diagnosed with primary or secondary T-cell lymphoma of the orbital and/or ocular adnexa over a period of 30 years. Primary outcome measures were age at diagnosis, affected site, type of T-cell lymphoma, treatment type, disease free survival and disease related death.
Results: Of the 14 patients included in the study, six were women and eight were men of which three were children. Mean age at diagnosis was 44. The most com- mon affected site was the orbit followed by the lacrimal sac, nasolacrimal duct, extraocular muscles and eyelids. Three cases were identified as primary orbital peripheral T-cell lymphoma. Of the secondary T-cell lymphomas the majority were associated with cutaneous primaries. Other types included anaplastic large cell and extranodal natu- ral killer nasal variants. Mean disease free survival was 57.2 months and eight (57%) patients died of lymphoma related causes.
Conclusion: This is the largest case series of orbital and ocular adnexal primary and secondary T-cell lymphoma described in the literature.