Choroidal and adnexal extranodal marginal zone B-cell lymphoma: presentation, imaging findings, and therapeutic management in a series of nine cases

Abstract

Purpose: To describe the clinical and imaging presentation, pitfalls in the diagnosis of choroidal extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT), as well as the therapeutic management and prognosis.

Methods: A retrospective case review of nine choroidal MALT lymphomas was performed. Initial clinical presentation and imaging findings of these histologically confirmed cases of lymphoma were analyzed. Treatment methods, time to diagnosis, systemic work-up, and treatment prognosis were assessed.

Results: Initial presentation was essentially blurred vision. The features described on examination were: anterior and posterior scleritis, iridocyclitis, choroidal infiltration, and exudative retinal detachment. Fluorescein and indocyanine green angiography as well as ultrasonography and optic coherence tomography provided arguments in favor of the diagnosis. Biopsy sites included conjunctiva, Tenon's capsule, deep scleral tissue, episclera, lacrimal gland, and choroid. Treatment mostly consisted of a combination of chemotherapy and radiotherapy. The mean time to diagnosis was 12 months.

Conclusions: Owing to the insidious onset of these tumors and their ability to simulate other conditions, the diagnosis is commonly delayed. The prognosis is generally good and treatment is effective in the case of localized lymphoma.

Figure 1

Fundus photography of bilateral choroidal involvement of choroidal MALT lymphoma. In the left eye (upper left): posterior choroidal infiltration. In right the eye (upper right): exsudative retinal detachment with peripheral cream-colored choroidal infiltrates. On the bottom left, green-light fundus photography of choroidal infiltrates; on the bottom right, same eye fluorescein angiography (1 : 02) showing the same features.

Figure 2

Upper left: fluorescein angiography (0 : 18). In this case, choroidal infiltration is more diffuse, and may be harder to identify; on the upper right, later time (6 : 06) of the same eye. On the bottom: B-Scan ultrasonography of a choroidal MALT lymphoma. Hypoechogenic and heterogenous parietal thickening, with no scleral breach.

Figure 3

Fluorescein angiography sequence showing unilateral macular choroidal neovascularization. Initial diagnosis was age-related macular degeneration.