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.
Rattunde et al. (Mon,) studied this question.