More than a masquerade syndrome: atypical presentations of vitreoretinal lymphomas

Abstract

Purpose: To present a population of patients sharing atypical manifestations of vitreoretinal lymphoma (VRL).

Methods: Institutional case series in a single tertiary center. Patients with cytologically proven VRL, referred between November 2009 and May 2010, were retrospectively reviewed. Diagnosis of VRL was based on cytology of vitreous samples, immunohistochemistry, and molecular biology. Patients with confirmed VRL and clinical features different from the typical manifestations were included. Demographical and clinical characteristics of these patients were studied.

Results: Twelve cases of VRL were diagnosed. Four cases (2M/2F) were considered atypical (A-VRL) in their presentations. Mean age for typical lymphoma (T-VRL) was 71.9 years (range: 62-87 years); mean age for A-VRL was 54 years (range: 50-59 years).

Conclusions: Diagnosis of VRL is challenging and diagnostic delay is frequent. The authors describe a series of patients sharing common characteristics, such as a younger age (p = 0.05), severe anterior chamber reaction, mild or no vitritis, and possible fulminant evolution.

Keywords: Acute retinal necrosis; intraocular lymphoma; masquerade syndrome; tuberculosis.