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Yoshiko Matsumoto, Atsushi Azumi, Azusa Akashi, Mari Sakamoto, Takayuki Nagai, Makoto Nakamura; Prognosis of lymphomas developed in the ocular adnexa: A single-center study. Invest. Ophthalmol. Vis. Sci. 2014;55(13):5441.
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To demonstrate a prognostic feature of patients suffering from lymphomas in the ocular adnexa.
A single-center retrospective study was conducted. Patients’ information was collected from the medical records of patients that underwent surgical resection of the lesions developed in the ocular adnexa, the conjunctiva, lid and orbit, and were diagnosed with lymphomas by pathological examination from April 1992 to June 2013. In all cases, Southern blot hybridization for immunoglobulin-gene rearrangement was performed to exclude non-lymphoma diseases such as IgG4-related ophthalmic disease. Flow cytometry analysis and G-banding were also done to make precise subtype diagnoses in accordance with WHO classification. For staging classification, systemic examinations were performed to evaluate metastasis. Kaplan-Meier method was used for survival rate analyses.
One hundred fifteen cases were diagnosed as lymphomas developed in the ocular adnexa. The median of patient age was 61 (range 10-87). The most frequent subtype was extranodal marginal zone B cell lymphoma of mucosa-associated lymphoid tissue type (MALT lymphoma), seen in 98 cases (85.2%). As for other subtypes of lymphoma, diffuse large B-cell lymphoma was seen in 6 cases (5.2%), follicular lymphoma in 5 (4.3%), and mantle cell lymphoma in 4 (3.5%). Affected sites in the ocular adnexa were orbit; 61 cases (53.0%), conjunctiva; 52 (45.2%), lid; 2 (1.7%). According to Ann-Arbor staging system, 75 cases (65.2%) were in stage I, 7 (6.1%) in stage II, 2 (1.7%) in stage III, and 15 (13.0%) in stage IV. In most of the cases, the disease was treated by either radiotherapy or chemotherapy, or both, but some cases were just observed after diagnosis. Systemic carcinoma development was observed in 14 cases (12.2%). The 10-year-overall-survival rate of all lymphoma cases was 94.0% and that of MALT lymphoma cases 94.8%. The cause-specific survival rates were 98.6% in all cases and 100% in MALT lymphoma cases. As for 10-year-non-recurrent rates in the cases of stage I or II, the rates were 64.6% in all cases and 67.6% in MALT lymphoma cases.
Most common subtype of lymphoma in the ocular adnexa is MALT lymphoma, and the 10-year-cause-specific survival rate was 100%. However, the non-recurrent rate was less than 70% and the development of cancer is not rare. The ocular adnexal lymphomas require long-term systemic follow-up.
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