Loading
7 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
A slow-growing MALT lymphoma of the tissues around the eye: the conjunctiva, the eye socket, the tear gland or the eyelid. It usually shows itself as a painless salmon-pink patch on the white of the eye or as an eye that has begun to bulge, and it is controlled in almost everybody, often by a short course of radiotherapy.
What it is. The tissues around the eye, the conjunctiva that lines the eyelids and covers the white of the eye, the orbit the eye sits in, the tear gland and the eyelids, are collectively the ocular adnexa. A marginal zone lymphoma arising there grows slowly and causes trouble by taking up space rather than by destroying anything. Three-quarters of lymphomas in this location are MALT lymphomas; the rest are follicular lymphoma, diffuse large B-cell lymphoma or others, which is why a biopsy is needed before anything is decided.
How it shows itself. A flat, salmon-pink patch under the conjunctiva that has been there for months; a painless swelling of the eyelid; an eye that has begun to bulge forward or to sit slightly out of line; double vision; a lump at the outer corner where the tear gland is. Pain, rapid growth and loss of vision are unusual and suggest a different, more aggressive lymphoma. Both sides are involved in a substantial minority, so the other eye is always examined.
| Setting | Approach | Guideline |
|---|---|---|
| Diagnosis and staging | A biopsy of the lesion is needed, because about a third of lymphomas at this site are not marginal zone lymphomas and the treatment differs. Imaging of the orbit, usually by magnetic resonance, maps the extent and the relationship to the optic nerve and the muscles. Staging of the rest of the body follows, because apparently localised ocular disease is sometimes part of a systemic marginal zone lymphoma. Both orbits are examined; bilateral disease is common and does not by itself mean the lymphoma has spread. | not mapped |
| Localised disease: radiotherapy, and an antibiotic where the evidence supports it | Radiotherapy to the orbit is the standard treatment for disease confined to the ocular adnexa and it controls it in almost everybody. In a series of 28 patients irradiated after a trial of doxycycline, 30 to 36 Gy in 15 to 18 fractions produced a response in all of them and a complete response in 89 per cent, with four-year progression-free survival of 74 per cent and little toxicity beyond three cases of grade 2 cataract and three of grade 1 blepharitis. Very low-dose schedules of about 4 Gy in two fractions are used to spare the lens and the tear gland, with retreatment if the lymphoma returns. Where local series have shown an association with Chlamydia psittaci, a course of doxycycline is a reasonable first step, and the same series shows that trying it first did not cost patients their response to radiotherapy afterwards. | not mapped |
| Disease beyond the orbit | Treated as any other marginal zone lymphoma: watched where it is causing no trouble, and treated with an anti-CD20 antibody alone or with chemotherapy when it is. The regimens, the thresholds for starting and the evidence are on the marginal zone and MALT lymphoma pages and in the treatment layer of this family. | not mapped |