Conjunctival Lymphoma Board Review
Master conjunctival lymphoma for NBEO and OKAP: salmon-pink lesions, MALT histology, biopsy handling and systemic staging.
Which clinical description is classic for conjunctival lymphoma?
Conjunctival Lymphoma in 30 Seconds
Conjunctival lymphoma is the most common malignant lymphoid tumor of the conjunctiva and usually represents an extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue, or MALT. Recognize the classic salmon-pink patch, obtain properly handled biopsy tissue and arrange systemic staging.
Classic Clinical Features
- Appearance: smooth, mobile, painless salmon-pink subconjunctival lesion, the classic salmon patch. Exam relevance: this is the description board questions almost always use.
- Location: often involves the fornix or bulbar conjunctiva. Exam relevance: a fornix lesion should raise suspicion.
- Symptoms: usually painless and slowly growing, sometimes asymptomatic. Exam relevance: chronic painless growth favors lymphoma.
- Laterality: may be unilateral or bilateral, and bilateral involvement is not uncommon. Exam relevance: bilateral disease does not exclude lymphoma.
- Histology: subepithelial growth, immunophenotyping typically shows CD20-positive B cells. Exam relevance: CD20 positivity supports a B-cell lymphoma.
- Most common type: extranodal marginal zone B-cell lymphoma of MALT. Exam relevance: MALT is the expected histologic answer.
Workup and Associations
- Biopsy: coordinate with pathology before biopsy so fresh tissue is submitted for flow cytometry, with additional tissue for histopathology and immunohistochemistry. Exam relevance: formalin-fixed tissue alone is insufficient for flow cytometry.
- Systemic workup: conjunctival disease can reflect systemic lymphoma. Exam relevance: do not stop after the ocular biopsy.
- MALT association: chronic antigenic stimulation may contribute, with a possible Chlamydia psittaci link in some populations. Exam relevance: the association varies by geography.
- Staging: confirmed cases require systemic staging because ocular adnexal lymphoma may represent primary disease or secondary systemic lymphoma. Exam relevance: every confirmed case needs systemic evaluation.
4 Facts Exams Always Ask
- A painless salmon-pink conjunctival patch is the classic presentation.
- MALT type extranodal marginal zone B-cell lymphoma is the most common type.
- Send fresh biopsy tissue for flow cytometry, not only formalin-fixed tissue.
- Perform systemic workup because conjunctival lymphoma may indicate systemic lymphoma.
The Classic Trap
Do not mistake a smooth salmon-pink patch for chronic conjunctivitis, reactive lymphoid hyperplasia, subconjunctival hemorrhage or an amelanotic conjunctival lesion without considering lymphoma. The second trap is specimen handling: fresh tissue for flow cytometry is essential. Any confirmed conjunctival lymphoma always requires systemic staging.
Clinical Pearl
The salmon-patch appearance and fresh tissue for flow cytometry are the two highest-yield board points. Most cases are low-grade B-cell MALT lymphomas with an excellent ocular prognosis, yet ocular adnexal lymphoma can herald or accompany systemic disease, so systemic involvement must always be excluded. External beam radiotherapy provides excellent local control for localized conjunctival MALT lymphoma.
Checkpoint 1
The most common subtype of ocular adnexal lymphoma is:
Checkpoint 2
For localized conjunctival extranodal marginal zone lymphoma, a commonly used treatment is:
An older adult presents with a painless, slowly enlarging salmon-pink conjunctival lesion in the inferior fornix. The lesion is smooth and mobile. There is no acute redness, discharge or pain. What diagnosis should be strongly suspected, and what are the next key steps in evaluation?
What is an important next step after diagnosing ocular adnexal lymphoma?
Sources & Publisher Info
References
- AAO BCSC Section 4: Ophthalmic Pathology and Intraocular Tumors.
- AAO BCSC Section 7: Oculofacial Plastic and Orbital Surgery.
- Shields CL, Shields JA. Clinical Ocular Oncology.
- AAO EyeWiki: Conjunctival Lymphoma.
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