Intraocular lymphoma

Superior vena cava syndrome is a set of symptoms resulting from partial or complete blockage of the superior vena cava. In the majority of cases, it develops secondary to lung tumors and lymphoma. It is characterized by edema of the head, neck, and upper limbs, large veins, and dyspnoea. Ophthalmological changes related to the syndrome rarely occur: eyelid swelling, conjunctival chemosis and suffusion have been described previously, and in one case, elevated episcleral pressure was also reported. Our 57-year-old female patient's superior vena cava syndrome was resolved with stent placement, and her small cell lung tumor was treated with chemotherapy...

Intraocular lymphoma (IOL) comprises a group of malignant tumours originating from lymphohematopoietic tissues that have a poor prognosis. These tumours predominantly occur in the vitreous and retina but are rarely found in the choroid. A few case reports and case series of choroidal lymphoma (CL) have been reported in the literature. CL is prone to misdiagnosis and incorrect treatment because it often mimics other intraocular diseases such as uveitis. This may seriously affect localisation of the primary lesion and delay treatment, which may even affect the patient's survival...

[This corrects the article DOI: 10.3389/fped.2022.982224.].

Methotrexate (MTX) may induce immunosuppression and facilitate the onset of lymphoproliferative disorders (LPD). Most cases of MTX-LPD occur in patients with rheumatoid arthritis; the incidence is high in Japan. Vitreoretinal lymphoma (VRL) is a rare non-Hodgkin's lymphoma that can masquerade as steroid-resistant chronic uveitis, leading to fatal diagnostic delay. A 68-year-old woman exhibited optic disc swelling and retinal vasculitis causing floaters in the right eye. She was undergoing long-term MTX treatment for rheumatoid arthritis; she previously had been diagnosed with MTX-LPD, which regressed upon discontinuation of MTX...

BACKGROUND: Intraocular lymphoma (IOL) is a masquerade syndrome that mimics uveitis, making diagnosis difficult. The serum soluble interleukin-2 receptor (sIL-2R), which is cleaved by matrix metalloproteinase (MMP) -2 and MMP-9, has been recognized as a tumor-related biomarker of malignant lymphomas. The aim of this study was to review the reliability of serum and vitreous sIL-2R for distinguishing IOL from uveitis. METHODS: Patients who underwent diagnostic vitrectomy for marked vitreous haze at Hokkaido University Hospital between April 2014 and June 2019 were enrolled...

The diagnosis of primary vitreoretinal lymphoma and central nervous system lymphoma is challenging. In cases with intraocular involvement, vitreous biopsy plays a pivotal role. Several diagnostic tests are employed to confirm a diagnosis and include cytologic evaluation, immunohistochemistry, flow cytometry, and cytokine analysis. The limitations of these conventional diagnostic tests stem from the often paucicellular nature of vitreous biopsy specimens and the fragility of malignant cells ex vivo. Several emerging molecular techniques show promise in improving the diagnostic yield of intraocular biopsy, possibly enabling more accurate and timely diagnoses...

OBJECTIVES: To report patients with systemic lymphoma and cytomegalovirus (CMV) retinitis, treated with a combination of oral and intravitreal antiviral agents on an outpatient basis. METHODS: Retrospective cases series. Information was gathered from the database of the Uveitis clinics at Moorfields Eye Hospital, United Kingdom from December 2014 to December 2018. The inclusion criteria comprised the diagnosis of systemic lymphoma, associated with a diagnosis of CMV retinitis...

Background: Ultra-low dose radiotherapy (ULDRT) (2 × 2 Gy) has been used for symptomatic control of low-grade lymphomas with surprising local control rates, suggesting that these entities could respond to lower doses. These are particularly desirable for the treatment of orbital sites and some publications refer to high rates of complete responses. In this paper, we present our experience with the use of ULDRT for indolent orbital lymphomas. Materials and methods: Electronic files and treatment plans of patients treated with ULDRT for low-grade orbital lymphoma were retrospectively reviewed...

BACKGROUND: Ocular mucosa-associated lymphoid tissue (MALT) lymphoma involving orbit and conjunctiva usually has an indolent clinical course with "salmon patch" mass as typical presentation. This study is to report a series of rare cases and investigate the clinical and pathological features of ocular MALT lymphoma that involved uveal tissue primarily and presented as posterior scleritis. METHODS: This retrospective, observational study was conducted at Beijing Tongren Hospital...

BACKGROUND: Vitreoretinal lymphoma (VRL) can commonly masquerade as chronic idiopathic uveitis due to its nonspecific clinical presentation. Thus, its early diagnosis is difficult. In this study, new logistic regression models were used to classify VRL and uveitis. Additionally, the diagnostic performance of interleukin (IL)-10, the IL-10/IL-6, and the Interleukin Score for IntraOcular Lymphoma Diagnosis (ISOLD) are evaluated. METHODS: Sixty-nine aqueous humors (AH) (46 VRL, 23 uveitis) and 65 vitreous humors (VH) (49 VRL, 16 uveitis) were collected from a single-center retrospective cohort...

Primary vitreoretinal lymphoma (PVRL) is a rare variant of primary central nervous system lymphoma (PCNSL) that presents diagnostic challenges. Here, we focused on circulating cell-free DNA (cfDNA) and interleukin-10 (IL-10) isolated from cerebrospinal fluid. Twenty-three VRL patients (17 PVRL, 2 PCNSL/O, and 4 relapsed VRL, from 10/2018 to 12/2021) and 8 uveitis patients were included in this study. CSF samples from 19 vitreoretinal lymphoma patients had sufficient cfDNA for next-generation sequencing. Of these patients, 73...

Purpose: To investigate the effect of systemic corticosteroids (CSs) on ocular hypertension (OHT) and to evaluate the management of OHT in children with non-Hodgkin lymphoma (NHL). Methods: Medical records of children with NHL treated in our institution between October 2016 and October 2019 were reviewed. The enrolled patients were divided into the mature B-cell lymphoma (MBL) group and lymphoblastic lymphoma (LBL) group based on pathology. Data on routine ophthalmic examinations and management of OHT were recorded...

Purpose: To report six Asian adult patients with retinoblastoma (RB). Design: Retrospective and observational small case series. Participants: Six patients with a white dome-shaped tumor of the retina were evaluated from May 10, 1995, to September 10, 2021. Main Outcome Measures: Initial tumor and associated fundus features, pathology, gene mutation, treatment, tumor course on follow-up, and salvage globe outcome. Results: The six affected Asian patients consisted of three men and three women...

The management of primary central nervous system (PCNSL) is one of the most controversial topics in neuro-oncology because of the complexity of the disease and the limited number of controlled studies available. In 2021, given recent advances and the publication of practice-changing randomized trials, the European Association of Neuro-Oncology (EANO) created a multidisciplinary task force to update the previously published evidence-based guidelines for immunocompetent adult patients with PCNSL and added a section on immunosuppressed patients...

To report a case of non-Hodgkin lymphoma (NHL) that was diagnosed 35-month of initial ocular manifestation. Retrospective chart review. A 53-year-old male presented with painless diminution of vision in both eyes. He subsequently underwent extensive laboratory investigations including multiple vitreous biopsies with a suspicion of intraocular lymphoma. Cytology from the vitreous aspirate failed to diagnose any relevant pathology. After 35-month from the onset of his ocular symptom, a brain biopsy revealed a round cell tumor suggestive of NHL...

Purpose: To seek novel diagnostic approaches, we improved the workflow of cell-free DNA (cfDNA) sequencing and evaluated its feasibility in vitreoretinal lymphoma (VRL) specimens; the profile of mutations was preliminarily analyzed for potential diagnostic value. Methods: The study was a diagnostic trial. 23 eyes of 23 patients with VRL and 25 eyes of 25 patients with inflammatory eye diseases were enrolled. Approximate 500μl undiluted vitreous humor and 10ml diluted vitreous fluid was obtained through diagnostic vitrectomy and sent for cytopathological examinations...

BACKGROUND: The classification of intraocular lymphomas is based on their anatomical location. They are divided into uveal lymphomas with involvement of the choroid, ciliary body or iris and vitreoretinal lymphomas with isolated or combined involvement of the vitreous body and/or retina. Over the last decades it has become increasingly possible to work out the clinical and pathobiological features of the various subtypes, thereby reducing the diagnostic hurdles and creating improved treatment options...

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BACKGROUND: Malignant lymphomas of the eye and its adnexal structures account for approximately 5-15% of extranodal lymphomas. According to anatomic and biological criteria, two large groups of lymphomas in and around the eye need to be distinguished: (1) primary lymphomas of intraocular structures and (2) primary lymphomas of ocular adnexa. Furthermore, there is a large spectrum of secondary manifestations of malignant lymphomas in ocular and periocular structures. OBJECTIVE: This article gives a summary of the classification and molecular pathology of various intraocular and periocular lymphomas as well as oncological systemic treatment with a focus on primary vitreoretinal lymphomas...

PURPOSE: To report an unusual case of unilateral anterior segment large B-cell intraocular lymphoma (IOL) presenting as a recurrent hypopyon anterior uveitis. CASE PRESENTATION: A 55-year-old female was referred because of recurrent unilateral anterior hypopyon uveitis with partial response to topical corticosteroid. All of the laboratory tests, review of systems and ocular sampling results were unremarkable. Given a high concern for masquerades syndromes, cytological specimens were obtained 3 times and the last sample showed large B cell lymphoma...