Vitreoretinal lymphoma: a 20-year review of incidence, clinical and cytologic features, treatment, and outcomes.
ABSTRACT OBJECTIVES To determine the incidence and clinical and cytologic diagnostic accuracy of vitreoretinal lymphoma (VRL) and to evaluate its clinical features, management, and outcomes in a cohort of patients who underwent diagnostic vitrectomy. METHODS Retrospective medical record review of 463 diagnostic vitrectomy specimens from 430 patients collected from October 1, 1990, through December 31, 2010, from Vancouver General Hospital and the British Columbia Cancer Agency. RESULTS A total of 22 patients were diagnosed as having VRL with a preoperative clinical diagnostic sensitivity of 77%, specificity of 73%, positive predictive value of 13%, and negative predictive value of 98%. The cytologic diagnostic sensitivity was 87% (27 of 31 specimens). The incidence of VRL in British Columbia doubled from 1990 to 2010, with a final incidence of 0.047 cases per 100 000 people per year. The mean age at diagnosis was 66 years. Seventeen patients (77%) were women. The initial diagnosis of lymphoma was VRL in 19 patients (86%), of whom 7 (37%) had concurrent central nervous system lymphoma. Recurrent disease was found in 11 patients. Large B-cell lymphoma was diagnosed in 20 patients (91%). The median progression-free survival was 11 months, and the median survival was 33 months from the initial diagnosis. CONCLUSIONS Vitreoretinal lymphoma remains a clinical diagnostic challenge. Early clinical suspicion with subsequent diagnostic vitrectomy for cytologic analysis and collaboration with the oncology department is critical to appropriate and prompt staging and treatment. More interdisciplinary studies are required to further characterize VRL and maximize the therapeutic options, thus improving the morbidity and mortality associated with the disease.
- SourceAvailable from: Daniela Alexandru-Abrams[Show abstract] [Hide abstract]
ABSTRACT: Primary intraocular lymphoma (PIOL) is a relatively rare form of Non-Hodgkin’s Lymphoma arising in the lymphoid tissues of the eye. It is highly correlated with primary CNS lymphoma (PCNSL) and it is estimated that up to 80% of patients presenting with PIOL will eventually manifest intracranial malignancy, which is the largest contributor to mortality. Most patients present with nonspecific visual symptoms, including floaters and blurry vision, and are often initially diagnosed with uveitis or retinitis. Definitive diagnosis requires biopsy of malignant tissues with demonstration of malignant lymphoid cells. Optimal therapy is to this point undefined, and the available literature is limited to case reports and retrospective series. Currently employed therapies include the use of localized external beam radiation therapy (EBRT), whole brain radiation therapy (WBRT), systemic chemotherapy, intrathecal chemotherapy, and, most recently, direct intravitreal (IVT) chemotherapy. While radiation therapy and chemotherapy can produce a high response rate, they have not been shown to effectively prevent relapse or the incidence of CNS spread. Methotrexate has been the most popular therapy used for the treatment of intraocular lymphoma. It has been administered systemically, intrathecally or intravitreally. However due to multiple mechanisms of resistance developed by lymphoma cells against methotrexate, this drug has been unable to prevent disease recurrence. The newest, and perhaps most promising, reported therapy includes the use of rituximab anti-CD20 monoclonal antibody either alone or in combination chemotherapy via intrathecal or IVT administration. Most cases in the literature employ combinations of available therapies, and there are no comparative studies of significant power to date. Multicenter collaboration will be required to determine the true relative efficacy and adversity of the therapeutic options available.
- Jama Ophthalmology 07/2014; 132(7):902-904. DOI:10.1001/jamaophthalmol.2014.419 · 3.83 Impact Factor
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ABSTRACT: Vitreoretinal lymphoma is a diffuse large B cell non-Hodgkin lymphoma. Targeting malignant cells with rituximab is being used increasingly as local chemotherapy, but information on this treatment is scant. We aimed to describe current therapeutic approaches, as well as responses to and complications of, intravitreal rituximab in patients with vitreoretinal lymphoma. Clinical data were collected in a standardised manner retrospectively on patients with vitreoretinal lymphoma treated with intravitreal rituximab. 48 eyes (34 patients) with vitreoretinal lymphoma were treated with a median of 3.5 intravitreal injections of rituximab (1 mg/0.1 mL) for new diagnosis (68.8%), progressive disease (29.9%) and maintenance therapy (2.1%). Intravitreal rituximab±methotrexate was the sole treatment in 19 eyes (39.6%). 31 eyes (64.6%) eyes achieved complete remission, after a median of 3 injections; 7 of these eyes developed recurrent disease. 11 eyes (22.9%) achieved partial remission. Although rituximab may have contributed to complications reported in 12 eyes (25.0%), a 2-line loss of Snellen visual acuity occurred in only 2 of those eyes (4.2%). Approaches in rituximab-based intravitreal chemotherapy vary widely, but our findings suggest that this treatment may be safe and effective in inducing remission in a majority of eyes with vitreoretinal lymphoma.The British journal of ophthalmology 10/2013; 98(1). DOI:10.1136/bjophthalmol-2013-304043 · 2.81 Impact Factor