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Area of Science:

  • Neuro-oncology
  • Ophthalmology

Background:

  • Primary central nervous system lymphoma (PCNSL) can present primarily in the eye (vitreoretinal lymphoma, VRL) or other central nervous system (CNS) compartments.
  • Ophthalmologists and neuro-oncologists may independently manage initial PCNSL presentations, necessitating shared understanding of ocular and non-ocular involvement.
  • Recent cohort studies offer updated insights into PCNSL's ocular manifestation patterns.

Purpose of the Study:

  • To review current prevalence rates of ocular involvement in PCNSL.
  • To analyze the timing of ocular involvement throughout the disease course.
  • To inform management practices for ophthalmologists and neuro-oncologists by clarifying PCNSL manifestation patterns.

Main Methods:

  • Analysis of published PCNSL cohort studies.
  • Stratification of cohorts based on primary site of involvement (non-ocular CNS vs. ocular).
  • Review of prevalence and timing of ocular and non-ocular CNS involvement.

Main Results:

  • In PCNSL cohorts with non-ocular CNS involvement, 10% had VRL at diagnosis and 16% at any time; exclusive eye disease was rare (<5%), with 5-9% secondary ocular involvement.
  • In VRL cohorts, 58% had a primary ocular diagnosis with a 50% risk of secondary non-ocular CNS involvement.
  • Rates of non-ocular CNS involvement in VRL cohorts were 41% at diagnosis and 69% over time.

Conclusions:

  • PCNSL presentation varies significantly, with distinct patterns of ocular and non-ocular CNS involvement depending on the initial site.
  • Awareness of these patterns is essential for timely diagnosis and effective management by both ophthalmologists and neuro-oncologists.
  • Further research can refine understanding and improve outcomes for patients with PCNSL and VRL.