Primary Intraocular Lymphoma: Rad-Path and Ophthalmologic Correlation

Denes Szekeres; Jonathan Parker; Evan Risch; Prasanna Vibhute; Girish Bathla; Shweta Agarwal; Amit Agarwal; Neetu Soni

doi:10.3174/ajnr.A8517

Primary Intraocular Lymphoma: Rad-Path and Ophthalmologic Correlation

AJNR Am J Neuroradiol. 2024 Sep 30:ajnr.A8517. doi: 10.3174/ajnr.A8517. Online ahead of print.

Authors

Denes Szekeres¹, Jonathan Parker¹, Evan Risch¹, Prasanna Vibhute¹, Girish Bathla¹, Shweta Agarwal¹, Amit Agarwal¹, Neetu Soni¹

Affiliation

¹ (1) University of Rochester, School of Medicine and Dentistry, Rochester NY 14620 (D.S), (2) Eastern Virginia Medical School, Norfolk, VA 23507 (J.P), (3) University of Connecticut, School of Medicine, Farmington, CT 06032 (ER), (4) Department of Radiology, Mayo Clinic, Jacksonville, 4500 San Pablo Road, Jacksonville, FL 55902, USA (P.V, A.A, N.S), (5) Department of Radiology, Mayo Clinic, 200 1st Street SW, Rochester, MN 55902, USA (G.B), (6) Department of Pathology, Mayo Clinic, Jacksonville, 4500 San Pablo Road, Jacksonville, FL 55902, USA (S.W).

PMID: 39349309
DOI: 10.3174/ajnr.A8517

Abstract

Primary intraocular lymphoma (PIOL) is a rare form of primary central nervous system lymphoma that poses diagnostic challenges due to its nonspecific clinical features and complex imaging characteristics. This paper presents a focus case and two companion cases, highlighting the complexities in identifying and treating PIOL. In the focus case, A 66-year-old male experienced gradual painless vision loss with choroidal thickening on funduscopic exam and subsequent follow-up MRI. Trans-vitreal biopsy confirmed PIOL, and the patient was treated with intravitreal steroids and systemic rituximab without recurrence. The 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 this 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's transformation. Enucleation was performed due to 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 gold 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 radiological features of PIOL, emphasizing the significance of diagnostic imaging in determining disease extent and guiding treatments.ABBREVIATIONS: CLL: chronic lymphocytic leukemia; CNS: central nervous system; FFA: fundus fluorescein angiography; IHC: cytometric immunohistochemistry; OCT: optical coherence tomography; FDG-PET/CT: Fluorodeoxyglucose positron emission tomography-computed tomography; PIOL: Primary intraocular lymphoma; PCNSL: primary central nervous system lymphoma; UBM: ultrasound biomicroscopy.