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

• References

• 1 Kalogeropoulos D, Vartholomatos G, Mitra A. et al. Primary vitreoretinal lymphoma. Saudi J Ophthalmol 2019; 33: 66-80
• 2 Riri K, Kalogeropoulos D, Mitsios A, Ploumi I, Androudi S. Intraocular Lymphoma. In: Interdisciplinary Cancer Research. Cham: Springer; 2022: 1-34
• 3 Levasseur SD, Wittenberg LA, White VA. Vitreoretinal lymphoma: a 20-year review of incidence, clinical and cytologic features, treatment, and outcomes. JAMA Ophthalmol 2013; 131: 50-55
• 4 Swerdlow SH, Campo E, Pileri SA. et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood 2016; 127: 2375-2390
• 5 Chan CC, Rubenstein JL, Coupland SE. et al. Primary vitreoretinal lymphoma: a report from an International Primary Central Nervous System Lymphoma Collaborative Group symposium. Oncologist 2011; 16: 1589-1599
• 6 Kalogeropoulos D, Asproudis I, Stefaniotou M. et al. The Large Hellenic Study of Uveitis: Diagnostic and Therapeutic Algorithms, Complications, and Final Outcome. Asia Pac J Ophthalmol (Phila) 2023; 12: 44-57
• 7 Yeh S, Weichel ED, Faia LJ. et al. 25-Gauge transconjunctival sutureless vitrectomy for the diagnosis of intraocular lymphoma. Br J Ophthalmol 2010; 94: 633-638
• 8 Jiang T, Zhao Z, Chang Q. Evaluation of cytologic specimens obtained during experimental vitreous biopsy using B-cell lymphoma line. Eur J Ophthalmol 2014; 24: 911-917
• 9 Bonzheim I, Sander P, Salmerón-Villalobos J. et al. The molecular hallmarks of primary and secondary vitreoretinal lymphoma. Blood Adv 2022; 6: 1598-1607
• 10 Nguyen DT, Houillier C, Choquet S. et al. Primary Oculocerebral Lymphoma: MTX Polychemotherapy Alone on Intraocular Disease Control. Ophthalmology 2016; 123: 2047-2050