Primary CNS lymphoma (PCNSL) can present with intraocular infiltration as the only site of disease — a distinct pattern known as primary vitreoretinal lymphoma (PVRL). Managing this presentation requires a tailored approach that accounts for the intraocular compartment and the patient's prior treatment history.
Intraocular infiltration can be the exclusive site of disease at presentation, referred to as primary vitreoretinal lymphoma (PVRL), or can occur as part of PCNSL with concomitant brain or meningeal involvement. When vitreoretinal disease is the sole manifestation, treatment decisions centre specifically on this ocular compartment.
For refractory or relapsed PVRL, the treatment approach is individualised based on patient characteristics and prior therapies received. Options span local ocular interventions, radiation-based strategies, and systemic approaches — the selection and sequence among these depends on specific clinical factors detailed in the full protocol.
DOI: 10.1093/neuonc/noac196
Intraocular infiltration can be the exclusive site of disease at presentation, referred to as primary vitreoretinal lymphoma (PVRL) or as a part of PCNSL with concomitant brain or meningeal disease.
Refractory and relapsed PVRL should be treated according to the patients' characteristics and prior treatments. Treatments include intravitreal injections of MTX, focal radiotherapy, WBRT, systemic chemotherapy, targeted treatment and HDC/ASCT (Good Practice Point).
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