[Ocular natural killer/T cell lymphoma: a clinicopathologic analysis].

Area of Science:

• Ophthalmology
• Oncology
• Pathology

Background:

• Ocular natural killer (NK)/T cell lymphoma is a rare malignancy.
• It can present as primary ocular disease or secondary to sinonasal or systemic involvement.
• The rarity and inflammatory presentation often lead to delayed diagnosis.

Purpose of the Study:

• To evaluate the clinicopathological features of ocular NK/T cell lymphoma.
• To analyze clinical data, morphology, immunophenotype, and outcomes of affected patients.

Main Methods:

• Retrospective analysis of 21 patients (22 eyes) with ocular NK/T cell lymphoma.
• Data collected included clinical presentation, radiological findings, pathological examination, and treatment outcomes.
• Immunophenotyping and Epstein-Barr virus-encoded RNA (EBER) in situ hybridization were performed.

Main Results:

• The study included 21 patients (10 male, 11 female) aged 3-77 years.
• Common presentations included eyelid swelling, decreased visual acuity, and proptosis; many were initially misdiagnosed as orbital pseudotumor or cellulitis.
• Pathological findings revealed atypical lymphoid infiltration with angioinvasive patterns; neoplastic cells were positive for cytoplasmic CD3ε, CD56, cytotoxic molecules, and EBER.
• Ten patients died within a mean of 6.3 months despite chemotherapy and radiotherapy.

Conclusions:

• Ocular NK/T cell lymphoma is a rare entity with both primary and secondary forms.
• The tumor's rarity and mimicry of inflammatory conditions complicate early diagnosis.
• The disease is characterized by high lethality and a poor prognosis, even with aggressive treatment.