Management of Bilateral Primary Vitreoretinal Lymphoma: A Case Report and Review of the Literature

Introduction

Intraocular lymphomas represent a diverse group of malignant lymphoid neoplasms, characterized by their heterogeneity. These neoplasms can be classified into two main categories: (1) those originating from the vitreoretinal tissue and (2) those that emerge within the uveal tract [1], [2]. The lymphomas affecting the retina and/or vitreous are considered primary tumors and are often associated with central nervous system (CNS) disorders [1], [2]. Primary vitreoretinal lymphomas (PVRLs) were previously referred to as primary intraocular lymphomas (PIOLs). PVRL predominantly involves the retina, vitreous body, and retinal pigment epithelium, whereas PIOL encompasses a broader range of intraocular structures, including the retina, vitreous body, choroid, and optic nerve, and can manifest as either vitreoretinal lymphoma or uveal/choroidal lymphoma. Both conditions share similar clinical presentations but differ in their specific intraocular involvement [1], [2]. Although rare, PVRLs constitute the most prevalent form of intraocular lymphoma. The estimated annual incidence is 0.46 per 100 000 individuals [1], [3]. This aggressive high-grade non-Hodgkin lymphoma is strongly associated with primary CNS lymphoma (PCNSL). According to the current World Health Organization (WHO) lymphoma classification, the majority of PVRL cases are categorized as diffuse large B-cell lymphomas [4]. Approximately 80% of PVRL patients will ultimately develop PCNSL, while 20% of PCNSL cases initially present with PVRL. Consequently, PVRL is typically fatal due to its eventual correlation with the CNS [5]. Despite its rarity, PVRL poses significant challenges in terms of diagnosis and treatment. The lack of effective therapeutic options and delays in diagnosis can contribute to a poor prognosis [1], [2].

Publication History

Received: 28 September 2023

Accepted: 01 June 2024

Article published online:
24 July 2024

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