Primary central nervous system lymphoma
ICD-10 C83.8 · ICD-11 2A81.5

Treatment of Primary CNS Lymphoma in Primary Vitreoretinal Lymphoma (Disease Limited to the Eye)

Clinical Scenario

This protocol addresses patients with primary vitreoretinal lymphoma (PVRL) — Primary CNS Lymphoma in which disease is limited to the eye, with vitreous and/or retinal involvement. When comprehensive staging excludes systemic organ involvement and CNS localisations beyond the eye, PVRL is the appropriate clinical designation. Patients in this scenario have no major comorbidities and are in good clinical condition.

Treatment Approach (Partial Overview)

Fit patients with PVRL are managed with a high-dose methotrexate-based induction chemotherapy regimen. Local intraocular therapy may be incorporated when rapid regression of intraocular disease is a priority.

Combination specifics, sequencing, consolidation strategy, and full eligibility criteria are in the structured protocol →

Treatment goal: Achievement of complete remission or partial response of intraocular disease.
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References

If comprehensive staging (see below) excludes the involvement of systemic organs and CNS localisations other than the eye, a diagnosis of primary vitreoretinal lymphoma (PVRL) should be considered.

Patients with no major comorbidities and good clinical condition.

Fit patients with PVRL should be treated with HD-MTX-based induction ChT, using the same combinations proposed for other patients with PCNSL. Intravitreal MTX injections can be added if a rapid regression of intraocular disease is needed.

DOI: 10.1016/j.annonc.2023.11.010

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