NK/T-cell Lymphoma With Orbital Involvement: A Case Report and Systematic Review of the Literature

Abstract

Purpose:To present the clinical course of a patient with recurrent NK/T-cell lymphoma (NKTL) involving the orbit and to review the literature on patients with NKTL involving the orbit.Methods:The PubMed database was searched for all cases of NKTL involving orbital, intraocular, or adnexal ocular structures.Results:Ninety-six patients were included in the final analysis. The mean age of diagnosis was 48.1 ± 16.8 years. The patients were 53/96 (55.2%) male and 43/96 (44.8%) female. Tumor location varied and included the orbit in 80/96 (83.3%), nasosinus in 56/96 (58.3%), uvea in 11/96 (11.5%), lacrimal gland in 9/96 (9.4%), lacrimal drainage system in 11/96 (11.5%), and conjunctiva in 7/96 (7.3%) cases. Management included surgical debulking in 29/96 (30.2%) cases, radiotherapy in 52/96 (54.2%) cases, and chemotherapy in 82/96 (85.4%) cases. Median survival was 6 months (95% CI: 5–9). Chemotherapy (hazard ratio = 0.80, 95% CI: 0.67–0.95,p= 0.013), radiotherapy (hazard ratio = 0.75, 95% CI: 0.64–0.87,p< 0.001), and orbital involvement being a recurrence of disease (hazard ratio = 0.79, 95% CI: 0.67–0.95,p= 0.009) were associated with improved survival. Advanced Ann Arbor stage (III–IV) at diagnosis (hazard ratio = 1.22, 95% CI: 1.08–1.38,p= 0.001), vision loss (hazard ratio = 1.18, 95% CI: 1.04–1.34,p= 0.009), proptosis (hazard ratio = 1.15, 95% CI: 1.01–1.30,p= 0.035) and periorbital swelling (hazard ratio = 1.15, 95% CI: 1.00–1.33,p= 0.048) were associated with poor survival.Conclusions:NK/T-cell lymphoma involving the orbit, globe, or ocular adnexa heralds a poor prognosis where early diagnosis and therapy are critical. The use of radiotherapy and chemotherapy is associated with improved survival.