A lymphoma can arise in conjunctival lymphoid follicles. Some conjunctival lymphomas are limited to the conjunctiva; others occur in conjunction with systemic malignant lymphoma. The majority of conjunctival lymphomas are monoclonal B-cell mucosa-associated lymphoid tissue (MALT) lymphomas. Conjunctival plasmacytoma, Hodgkin lymphoma, and T-cell lymphomas are less common.
Conjunctival lymphoma has essentially the same clinical appearance as benign lymphoid hyperplasia. It appears as a salmon-pink, mobile mass on the conjunctiva (Fig 12-16). The lesions are usually unilateral; 20% are bilateral. A diffuse lesion may masquerade as chronic conjunctivitis. An epibulbar mass fixed to the underlying sclera may be a sign of extrascleral extension of uveal lymphoid neoplasia. Most patients with conjunctival lymphoma are either older than 50 years or immunosuppressed.
LABORATORY EVALUATION AND MANAGEMENT
Patients with conjunctival lymphoma should be referred to an oncologist for systemic evaluation because underlying systemic lymphoma may be present or may eventually develop in up to 31% of these patients. Unless a tumor is small enough to be removed completely, incisional biopsy is indicated for histologic diagnosis. Local external-beam radiation therapy is usually curative for lesions confined to the conjunctiva, but systemic chemotherapy is required for the treatment of systemic lymphoma. More recently described treatments for conjunctival MALT lymphoma include intralesional interferon-α2b and intralesional or systemic rituximab.
Figure 12-16 Conjunctival lymphoma.
Blasi MA, Tiberti AC, Valente P, et al. Intralesional interferon-α for conjunctival mucosa-associated lymphoid tissue lymphoma: long–term results. Ophthalmology. 2012;119(3): 494–500.
Raderer M, Kiesewetter B, Ferreri AJ. Clinicopathologic characteristics and treatment of marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). CA: A Cancer Journal for Clinicians. 2016;66(2):153–171.
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Warner MA, Stagner AM, Jakobiec FA. Subepithelial tumors of the conjunctiva. In: Mannis MJ, Holland EJ, eds. Cornea. Vol 1. 4th ed. Philadelphia: Elsevier; 2017:442–465.
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.