CASE REPORT article
Front. Ophthalmol.
Sec. Neuro-Ophthalmology Disorders
Triple-Hit Diffuse Large B-Cell Lymphoma with Choroidal and Cavernous Sinus Involvement Mimicking Inflammatory and Neuro-ophthalmic Disease: Case Report
Provisionally accepted- 1Department of Ophthalmology, Universidad de Puerto Rico Recinto de Ciencias Medicas, San Juan, Puerto Rico
- 2Department of Pathology, Universidad de Puerto Rico Recinto de Ciencias Medicas, San Juan, Puerto Rico
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Background To describe our findings in a rare case of secondary triple-hit diffuse large B-cell lymphoma (DLBCL) with choroidal and central nervous system (CNS) involvement, presenting with bilateral serous retinal detachments (SRD) and ophthalmoplegia. Case presentation A 44-year-old male presented with a 4-month history of bilateral vision loss, right-sided headaches radiating to the periorbital area, and oral numbness within the mental nerve distribution. Review of systems was notable for unintentional 25-pound weight loss and chronic back pain. Corrected visual acuities were 20/150 in the right eye and 20/50 in the left. External exam was remarkable for right-sided ptosis, diminished pupillary light response without afferent pupillary defect, and limitation of extraocular movements in all gazes. Fundus examination showed bilateral, multifocal SRD with associated increased choroidal thickness. Systemic workup showed right-sided prominence of the cavernous sinus, heterogeneous bone marrow signal throughout the spine, splenomegaly, retroperitoneal lymphadenopathy, and a hypodense hepatic lesion. Cerebrospinal fluid analysis revealed elevated white blood cell count and protein concentration. A lymph node biopsy revealed DLBCL with Bcl6, Bcl2, and c-Myc rearrangements. Treatment with combined intrathecal and systemic chemotherapy resulted in significant improvement in both systemic and ocular symptoms. Conclusion This case underscores the importance of considering secondary lymphoma in patients presenting with bilateral SRD and neuro-ophthalmic deficits, even in the absence of known systemic malignancy. The combination of cavernous sinus syndrome with concomitant mandibular nerve involvement should prompt CNS and systemic evaluation for hematologic malignancy. Patients with triple-hit DLBCL phenotype may achieve dramatic visual recovery following modern targeted chemoimmunotherapy.
Keywords: Cavernous sinus syndrome, Diffuse large B-cell lymphoma, Ophthalmoplegia, serous retinal detachments, triple-hit
Received: 28 Dec 2025; Accepted: 15 Feb 2026.
Copyright: © 2026 Nieves-Rios, Feneque González, Rivera Rolon, Rodriguez-Padilla, Villegas and Serrano. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
* Correspondence: Christian Nieves-Rios
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