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Steve D. Levasseur, Leah A. Wittenberg, Valerie A. White; Primary Intraocular Lymphoma: A Twenty-year Review Of Incidence, Clinical Features, Treatment And Outcomes. Invest. Ophthalmol. Vis. Sci. 2012;53(14):5615.
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© ARVO (1962-2015); The Authors (2016-present)
To determine the incidence, clinical and cytological diagnostic accuracy of primary intraocular lymphoma (PIOL), and to evaluate its clinical features, management and outcomes in a cohort of patients who underwent diagnostic vitrectomy.
A retrospective chart and database review of 462 diagnostic vitrectomy specimens from 429 patients collected between October 1990 and December 2010 from the Vancouver General Hospital and the British Columbia Cancer Agency was performed. Vitreous specimens were obtained and prepared in a standardized manner.
A total of 21 patients were diagnosed with PIOL with a preoperative clinical diagnostic sensitivity of 76%, specificity of 73%, positive predictive value of 13% and negative predictive value of 98%. The incidence of PIOL in British Columbia increased by a factor of 1.5x between 1990 and 2010, with a final incidence of 0.038 cases/100 000 people/ year. The median age at diagnosis was 69 years. Seventeen (81%) were female. All were immunocompetent. The initial diagnosis of lymphoma was PIOL in 18 (86%) of patients, of which 7 (39%) had concurrent central nervous system lymphoma (CNSL). The remaining 3 (14%) had primary CNSL and subsequently developed PIOL . Recurrence of disease after successful treatment occurred in 11 patients, one of whom had two recurrences and another three. The anatomical sites involved were; 9 eyes (64%), 4 CNS (29%) and 1 pulmonary (7%). Large B-cell type lymphoma was diagnosed in 19 (90%) with the remainder being T cell type. The median progression-free survival was 11 months and the median survival 33 months from the initial diagnosis.
PIOL remains a clinical diagnostic challenge and continues to carry a poor prognosis despite several advancements in treatments. Early clinical suspicion, with subsequent diagnostic vitrectomy for cytological analysis, and collaboration with oncology is critical to promptly offer appropriate staging and treatments to the patient. More interdisciplinary studies are required to further characterize PIOL and maximize the therapeutic options to improve both the morbidity and mortality associated with the disease.
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