Primary intraocular lymphoma (PIOL) is a rare and highly aggressive tumor, which tends to mimic symptoms and findings of uveitis leading to the delay in its diagnosis. The purpose of this report is to detail the case of a patient who presented with unilateral blurring of vision clinically treated as PIOL and to present the therapeutic challenges in patients without biopsy-proven PIOL.
This report details a 61-year-old Filipino male who presented with unilateral blurring of vision and prominent subretinal lesions with the characteristic “leopard-skin” appearance. Anterior and posterior segment findings were suggestive of unilateral panuveitis with high suspicion for PIOL. Immediate systemic work-up and diagnostic vitrectomy was performed yielding unremarkable results for both infectious and malignant etiologies. Due to high clinical suspicion for PIOL, local chemotherapy with intravitreal methotrexate was initiated. Subsequent clinical improvement of ocular lesions was observed.
During the course of local chemotherapy, an intranasal mass developed, and tissue biopsy confirmed the diagnosis of Extranodal natural killer/T-cell lymphoma. Chemoradiotherapy was then initiated.
Diagnosis of primary intraocular lymphoma remains challenging because of the high false negative rates of diagnostic vitrectomy and the unavailability of adjunctive examinations in the Philippines, such as vitreous interleukin analysis and gene rearrangement studies, resulting in the delay in initiation of treatment. Current therapeutic approaches are centered in the local control of the ocular lesions and the prevention of disease progression and extraocular spread. Despite the poor prognosis of this disease, prompt diagnosis and initiation of treatment has been proven to improve overall survival rate.