Primary Intraocular Lymphoma: Rad-Path and Ophthalmologic Correlation

SUMMARY:

Primary intraocular lymphoma (PIOL) is a rare form of primary central nervous system lymphoma that poses diagnostic challenges because of its nonspecific clinical features and complex imaging characteristics. This paper presents a focus case and 2 companion cases, highlighting the complexities in identifying and treating PIOL. In the focus case, a 66-year-old man experienced gradual painless vision loss with choroidal thickening on funduscopic examination and subsequent follow-up MRI. Transvitreal biopsy confirmed PIOL, and the patient was treated with intravitreal steroids and systemic rituximab without recurrence. Companion case 1 involved a 66-year-old woman with vision changes and choroidal thickening with episcleral extension on MRI suggestive of intraocular lymphoma and ultimately treated with radiation with the presumed diagnosis of PIOL. In the companion case 2, a 63-year-old man with ocular symptoms was diagnosed with chronic lymphocytic leukemia along with vitreoretinal Richter transformation. Enucleation was performed because of a lack of visual potential and failure of chemotherapy, which confirmed PIOL. Distinguishing PIOL from other ocular conditions is crucial, given its potential for CNS involvement. Imaging plays a vital role in corroborating clinical findings. While cytology remains the standard for diagnosis, supplementary tests, including cytokine analysis, immunohistochemistry, and flow cytometry, provide additional insights. PIOL treatment strategies are tailored to disease extent, ranging from locoregional chemotherapy to invasive enucleation. CNS involvement carries a poor prognosis and must evaluated and surveilled with MRI. In conclusion, this case series reviews the clinical and radiologic features of PIOL, emphasizing the significance of diagnostic imaging in determining disease extent and guiding treatments.