Primary intraocular lymphoma: an International Primary Central Nervous System Lymphoma Collaborative Group Report

Abstract

Background: Primary intraocular lymphoma (PIOL) is an uncommon subset of primary central nervous system lymphoma. Because it is rare and difficult to diagnose, the natural history and optimal management are unknown.Patients and methods: A retrospective study of 83 HIV negative, immunocompetent PIOL patients was assembled from 16 centers in seven countries.Results: Median age at diagnosis was 65. Median ECOG performance status was 0. Presenting symptoms included blurred vision, decreased visual acuity, and floaters. Median time to diagnosis was 6 months. Diagnosis was made by vitrectomy (74), choroidal/retinal biopsy (6) and ophthalmic exam (3). Eleven percent had positive CSF cytology. Initial treatment was categorized as focal in 23 (intra-ocular methotrexate, ocular radiotherapy) or extensive in 53 (systemic chemotherapy, whole brain radiotherapy). Six received none; details are unknown in one. Forty-seven relapsed: brain 47%, eyes 30%, brain and eyes 15%, and systemic 8%. Median time to relapse was 19 months. Focal therapy alone did not increase risk of brain relapse. Median progression free (PFS) and overall survival (OS) were 29.6 and 58 months, respectively, and unaffected by treatment type.Conclusion: Treatment type did not affect relapse pattern, median PFS or OS. Focal therapy may minimize treatment toxicity without compromising disease control.

The evaluation and treatment of primary intraocular lymphoma

“…Generally, the prevailing consensus is to employ local therapies for restricted ocular disease (PIOL) and to reserve systemic therapy for those with CNS disease [1,15,17]. Local therapy is typically considered the intervention of choice when thorough neurologic workup is non-revealing in light of a histological diagnosis of intraocular lymphoma [17].…”

“…Primary intraocular lymphoma (PIOL), also known as primary vitreoretinal lymphoma (PVRL), is a rare subset of primary central nervous system lymphoma (PCNSL) arising in the retina, vitreous, subretinal pigment epithelium, or optic nerve head [1]. Intraocular lymphoma can arise primarily within the lymphoid tissues of the eye or due to metastatic spread of hematopoietic malignancy from the CNS.…”

“…The most novel therapy for the treatment of PIOL involves the use of rituximab, a humanized antibody targeting CD20+ B cells, in either IV or intravitreal administration [13,14]. Because several treatment modalities have been reported in the literature, many authors have called for further research into the comparative effectiveness of the available treatments and the establishment of treatment guidelines thereof [1,15,16]. This paper provides an up-to-date review of the multiple interventions currently employed in PIOL management, and to our knowledge contains the most comprehensive meta-analysis of case reports and retrospective series of novel therapies to date.…”

Treatment approaches for primary CNS lymphomas

“…As a consequence of these difficulties to achieve therapeutic drug concentrations into the eyes, ocular failure is common. Good rates of ocular disease control combining ocular irradiation to MTX-based chemotherapy have been reported [61,62]. Promising results seem to derive from the intraocular administration of methotrexate by injections [63,64].…”

Combined therapy associating systemic platinum-based chemotherapy and local radiotherapy into the treatment of primary intraocular lymphoma

“…PIOL is considered to be a rare subtype of central nervous system lymphoma with a high risk of relapse that is characterised by either intraocular relapse or metastasis into the central nervous system (1,7). This type of lymphoma is mainly found in elderly and immunocompromised patients and rarely in young patients (2,3).…”