Conjunctival lymphoma
ICD-10 C69.0 · ICD-11 2B33.5&XA8PS3

Treatment of Biopsy-Proven Conjunctival EMZL with Unilateral Disease and No Systemic Involvement

This page summarises the clinical scenario and outlines the general treatment approach for patients with histologically confirmed conjunctival extranodal marginal zone B-cell lymphoma (EMZL) confined to one eye, with staging showing no systemic disease.

Clinical Scenario

The diagnosis is established by incisional biopsy, which is the gold standard for histopathological and cytological confirmation. Disease is limited to the conjunctiva of one eye with no evidence of systemic lymphomatous involvement. For elderly or frail patients with minimal symptoms in the setting of unilateral disease, a watchful waiting strategy may also be considered.

Treatment Approach (First-Line)

For patients where systemic treatment is indicated, the protocol involves chemotherapy — including single-agent and combination regimens. The specific choice of agent or regimen, eligibility criteria, and full treatment algorithm are detailed in the complete protocol.

Full regimen, sequencing, and dosing available via the link below.
Instant Access to Structured Evidence-Based Regimens
References

DOI: 10.1038/s41433-022-02176-2

The gold standard of conjunctival lymphoma diagnosis is an incisional biopsy for histopathological and cytological examination.

A "wait and watch" strategy has been proposed for elderly or frail patients with little symptoms, particularly in the setting of unilateral disease.

Further, in patients with high grade localised disease, systemic chemotherapy may also be considered if there are contraindications to, intolerance or poor response to first line EBRT.

The standard single-agent chemotherapy for non-Hodgkin's lymphoma remains chlorambucil, whereas combination therapy used includes cyclophosphamide, vincristine and prednisone (CVP), and cyclophosphamide, hydroxydaunorubicin, vincristine and prednisone (CHOP).

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