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Patrick T Logan, Denise Miyamoto, Yasser Althnayan, Dana Alolayet, Jaazeel Mulla, Miguel N Burnier; Ocular adnexal lymphoma: an 11-year retrospective study of different subtypes and their clinical implications. Invest. Ophthalmol. Vis. Sci. 2017;58(8):1799. doi: https://doi.org/.
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© ARVO (1962-2015); The Authors (2016-present)
Ocular adnexal lymphoma (OAL) is a malignancy with increasing incidence rates, which can be subdivided into primary extranodal lymphoma in the conjunctiva and orbit (POL) or ocular involvement of a systemic lymphoma (SOL). This retrospective study aimed to compare the epidemiology, clinical features, and prognosis of OAL, POL, and SOL.
We performed a review of 40 patients with OAL diagnosed between 2006 and 2016 at the Ocular Pathology Laboratory. We evaluated demographic variables, lactate dehydrogenase (LD) levels, treatment modality and response, and follow-up.
Of the 40 patients diagnosed with OAL, 22 were treated and had complete demographic and clinical information. Of these, 16 patients had POL while 6 had SOL. The average age was similar in both groups (69.2 vs. 68.7), with equal gender distribution for POL (8F/8M) and a female predominance for SOL (5F/1M). Single lesions were located in the orbit (POL=8; SOL=4) and conjunctiva (POL=7; SOL=1); multifocal tumors occurred in 2 patients with POL and 1 patient with SOL. Bilateral involvement occurred in 2 POL and 1 SOL patient. Symptoms were more prominent in POL (11; 68.8%) than in SOL (2; 33.3%). Lymph node enlargement was present in 2 cases of SOL. Three POL and 1 SOL patients had abnormal LD levels. Among all OAL, extranodal marginal zone lymphoma MALT lymphoma (EMZL) was the most frequent subtype (POL=11; SOL=1) followed by follicular lymphoma (POL=2; SOL=3). One POL patient had diffuse large B-cell lymphoma and 2 SOL patients were diagnosed with mantle cell lymphoma. Whereas the predominant treatment modalities were radiotherapy (n=11), surgery (n=3), chemoradiotherapy (n=1) in POL, chemotherapy (n=3) and immunotherapy (n=1) were the prevailing therapies in SOL. In 4 patients, treatment modality was not available. Mean follow-up in POL was 47.9 months with 2 recurrences (1 EMZL; 1 FL) and 1 death (EMZL) patient, while mean follow-up in SOL was 36.3 months (FL) with no deaths.
POL was the most frequent type of OAL with similar gender distribution, more symptomatic presentation and radiotherapy was the predominant treatment. Conversely, SOL had a female predilection with signs of systemic involvement (lymphadenomegaly), more discrete ocular symptoms, and required systemic therapy. These differences highlight the importance to rule out systemic involvement in OAL.
This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.
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