Conjunctival Lymphoma--An International Multicenter Retrospective Study

Area of Science:

• Ophthalmology
• Oncology
• Hematology

Background:

• Conjunctival lymphoma (CL) is a rare malignancy with diverse subtypes.
• Previous evaluations of clinical features across CL subtypes in large cohorts are limited.
• Understanding subtype-specific characteristics is crucial for accurate diagnosis and prognosis.

Purpose of the Study:

• To characterize the subtype-specific clinical features of conjunctival lymphoma (CL).
• To evaluate the impact of these distinct subtypes on patient outcomes.
• To provide a comprehensive analysis of a large cohort of CL patients.

Main Methods:

• Retrospective multicenter study involving seven eye cancer centers.
• Data collected from 268 conjunctival lymphoma (CL) patients diagnosed between 1980 and 2010.
• Analysis of overall survival, disease-specific survival, and progression-free survival.

Main Results:

• Two hundred sixty-three patients with B-cell non-Hodgkin lymphoma were included.
• Extranodal marginal zone lymphoma (EMZL) was the most frequent subtype (68.4%), followed by follicular lymphoma (FL) (16.3%), mantle cell lymphoma (MCL) (6.8%), and diffuse large B-cell lymphoma (DLBCL) (4.6%).
• MCL and DLBCL showed a significantly poorer prognosis (5-year disease-specific survival of 9.0% and 55.0%, respectively) compared to EMZL (97.0%) and FL (82.0%). MCL frequently presented as secondary disease, stage IVE, and bilateral manifestation.

Conclusions:

• Conjunctival lymphoma comprises four main B-cell non-Hodgkin lymphoma subtypes: EMZL, FL, MCL, and DLBCL.
• Histological subtype is a primary predictor of patient outcome, with MCL and DLBCL indicating a worse prognosis.
• MCL exhibits unique characteristics, including a high incidence of secondary disease, advanced stage, and bilateral involvement.