Primary Orbital Extranodal NK/T-Cell Lymphoma: A European Case Report

Purpose: Extranodal natural killer/T-cell lymphoma, nasal type (ENKTL), is a rare and aggressive malignancy. Primary orbital involvement is exceptionally uncommon. We report a case of orbital ENKTL in a White patient presenting with rapidly progressive orbital symptoms. Observations: A 69-year-old woman presented with eyelid swelling, diplopia, and exophthalmos. An initial diagnosis of conjunctivitis by a community ophthalmologist proved unresponsive to treatment, prompting referral to a tertiary eye center. Orbital magnetic resonance imaging revealed a retrobulbar mass. Biopsy confirmed Epstein-Barr virus-associated ENKTL, and staging excluded systemic disease. The patient received age-adjusted modified SMILE (mSMILE) chemotherapy (dexamethasone, methotrexate, ifosfamide, L-asparaginase, etoposide) followed by concurrent platinum-based chemoradiotherapy. She achieved complete remission and reported substantial improvement in quality of life. Conclusions and Importance: Even exceptionally rare orbital lesions should be considered in cases of rapidly progressive orbital disease. Early imaging, appropriate biopsy, and multidisciplinary management are essential to achieve optimal outcomes.