Orbital lymphoma masquerading as panuveitis

Abstract

Purpose: To describe a case of orbital mucosa-associated lymphoid tissue (MALT) lymphoma masquerading as unilateral panuveitis.

Methods: Retrospective chart review.

Results: A 53-year-old female patient with unilateral vitritis and exudative retinal detachment refractory to immunosuppressive treatment was eventually diagnosed with orbital MALT lymphoma. Following treatment with radiotherapy and rituximab, the patient's intraocular inflammation and retinal detachment resolved.

Conclusions: Orbital MALT lymphoma can masquerade as refractory unilateral panuveitis with exudative retinal detachment and appears to respond to a combination of radiotherapy and specific B-cell-targeted systemic therapy.

FIGURE 1

Fundus photographs and OCT images before and after treatment for MALT lymphoma. Initial examination showed a depigmented fundus with optic nerve head swelling (A) and macular edema on OCT (B). Following treatment, as the serous RD resolved the fundus appeared more depigmented, accompanied with pigmentary deposits in the macula, but the optic nerve swelling and macular edema improved (C, D).

FIGURE 2

MRI brain/orbits and histopathology of orbital MALT lymphoma. T1-weighted axial images with fat suppression and gadolinium demonstrated abnormal enhancement within the orbit, along the posterior sclera and intraorbital optic nerve (A). Photomicrograph of orbital MALT lymphoma demonstrates many small round and cleaved atypical lymphoid cells surrounding the nerve (B).