Primary Ocular Natural Killer/T-Cell Lymphomas: Clinicopathologic Features and Diagnosis

PURPOSE: Primary ocular natural killer/T-cell lymphoma (NKTL) is rare. This study aims to characterize the clinical manifestations, diagnosis and treatment methods of primary ocular NKTL. METHODS: Clinical data of seven patients with ocular NKTL from 1982 to 2004 were collected. A morphologic, immunohistochemical and molecular analysis was undertaken. RESULTS: Clinical manifestations and computed tomography of the patients were typical. LCA CD56 CD3epsilon Ki-67 granzyme B (GrB) and T-cell intracellular antigen-1(TIA-1) were mostly positive. Clonal T-cell-receptor gene rearrangements of five patients showed negative findings, and the detection of Epstein-Barr virus showed positive. All patients died 5 months to 28 months after presentation. CONCLUSION: Ocular NKTL is a highly aggressive clinical course with poor prognosis. The diagnosis relies on clinicopathologic findings.