Clinical features, outcomes of eyelid lymphoma
Lymphoma of the eyelid mostly comprises several lymphoma subtypes, with histological subtype being the main predictor of outcome, according to a study.
Specifically, the lymphoma subtypes include extranodal marginal zone lymphoma (EMZL), follicular lymphoma (FL), diffuse large B-cell lymphoma (DLBCL), mantle cell lymphoma (MCL) and mycosis fungoides (MF). Secondary eyelid lymphomas frequently occur as high-grade DLBCL and MCL as well as MF. Among these subtypes, EMZL, FL and MF were associated with significantly better prognosis compared with MCL and DLBCL.
The retrospective observational case series included 86 patients (mean age 63 years; 55 percent male) with primary or secondary lymphomas affecting the eyelid and who presented to seven international eye cancer centres from 1980 through 2015. The main endpoints investigated were overall survival, disease-specific survival (DSS) and progression-free survival.
Of the patients, majority (83 percent) had non-Hodgkin B-cell lymphomas, whereas 17 percent had T-cell lymphomas. EMZL was the most common subtype, accounting for 37 percent of the cohort. This was followed by FL (23 percent), DLBCL (10 percent), MCL (8 percent) and MF (9 percent). Meanwhile, secondary lymphomas frequently consisted of MCL (57 percent), DLBCL (56 percent) and MF (88 percent.
There was a female predominance for EMZL (69 percent), while MCL and MF were more prevalent in males (71 and 88 percent, respectively). Commonly used treatment was external beam radiation therapy for localized EMZL and FL, and chemotherapy for DLBCL, MCL and high Ann Arbor stage EMZL and FL. The corresponding 5-year DSS for EMZL, FL and MF was 88, 88 and 86 percent compared with only 21 and 50 percent for DLBCL and MCL.
Ocular lymphoma occurs as either intraocular or orbital and adnexal, and commonly caused by non-Hodgkin lymphoma. Patients with this disease often have decreased vision with nonresolving uveitis. Ocular lymphoma may be identified by obtaining an intraocular biopsy specimen, with chemoradiation being the most effective treatment, although its use is associated with significant ocular and cerebral morbidity. [Roque R, et al. “Ocular Lymphoma”. Medline: 2016]