Primary Vitreoretinal Lymphoma

A 60-year-old female patient with bilateral intermediate uveitis and a history of previous episodes of anterior uveitis underwent extensive systemic workup for an underlying cause with no positive results and was treated conservatively with oral corticosteroids. She responded optimally to the treatment but recurred with progressively worsening episodes of ocular inflammation. Considering the bilateral symmetric nature of the disease and repeated negative systemic workup, the dilemmas were galore, and the patient was advised vitreous biopsy. Cytological and histopathological examinations of the vitreous specimens of both eyes were inconclusive. Yet another recurrence, but this time with a yellowish-white retinal lesion exhibiting characteristics of lymphoma on imaging, brought the provisional diagnosis of masquerade syndrome right to the top. Head MRI and CSF examination confirmed a primary vitreoretinal lymphoma with CNS involvement, a notorious masquerade with progressively worsening ocular inflammation and a high false-negative yield on vitreous biopsy. A strong clinical suspicion with a timely meticulous biopsy and oncology consult may not just be sight-saving but lifesaving in some of these cases.