Background Flashcards
Under what type of lymphomas are intraocular/orbital lymphomas classified?
Intraocular/orbital lymphomas are classified as NHLs (B-cell histology > T-cell histology).
What is the median age of onset? What are the 2 main types of eye lymphoma?
The median age of onset is 50–60 yrs (females > males). The 2 main types are intraocular (bimodal age distribution at 31 and 60 yrs) and orbital/ocular adnexal lymphoma (median age at 65 yrs).
What are the most common histologies?
Intraocular: usually diffuse large B-cell lymphoma
Orbital: extranodal marginal zone B-cell lymphoma or MALT lymphoma
Which structures are involved in intraocular vs. orbital lymphomas?
Intraocular lymphomas involve the neural structures.
Orbital lymphomas can involve the conjunctiva, ocular adnexa, lacrimal apparatus, uvea, and retrobulbar areas.
What % of pts with primary intraocular lymphomas develop CNS Dz within 3 yrs?
60%–80% of pts with intraocular lymphomas develop CNS Dz.
What % of pts with primary CNS lymphomas develop intraocular involvement?
25% of pts with primary CNS lymphoma develop intraocular involvement.
What is the recurrence rate after Tx of intraocular lymphomas?
The recurrence rate after Tx is ∼50%.
Intraocular lymphoma is bilat in what % of pts? How about orbital/adnexa lymphoma?
Intraocular: 80%
Orbital: 20%
What is a common chromosomal translocation in intraocular lymphoma? What % of pts have this translocation?
t(14;18) is a common translocation in intraocular lymphoma. 56% of pts have this translocation.
Ocular lymphoma cells are usually positive for which important immunohistochemical markers?
Ocular lymphomas usually stain positive for CD20 and bcl-2, bcl-6, and MUM1.
What type of lymphoma accounts for most lymphomas of the ocular adnexa?
MALT lymphoma accounts for most lymphomas of the ocular adnexa.
Which ocular lymphoma has a male predominance and is associated with mycosis fungoides?
Ocular lymphoma of T-cell histology occurs predominantly in males and is associated with mycosis fungoides.
With what infectious agent has orbital MALT been associated? What site is often involved?
Chlamydia psittaci has been associated with orbital MALT lymphomas. The lacrimal gland is often involved. Tx with doxycycline alone in a phase II trial of 47 pts (89% with biopsy + Chlamydia DNA) resulted in a 5-yr PFS of 55% and lymphoma regression in 65% of pts. (Ferreri AJ et al., JCO 2012)
Lymphoma of what orbital structure has a high propensity for LN spread?
Lacrimal gland lymphoma has a high propensity for LN spread (>40% are LN+).
Which lymphomas have a better prognosis: orbital or intraocular?
Orbital lymphomas tend to be indolent, whereas intraocular lymphomas are aggressive with a high propensity for CNS involvement.
