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Primary CNS lymphoma with intraocular involvement: International PCNSL Collaborative Group Report
S A Grimm1, C A McCannel, A M P Omuro
1Department of Neurology, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.
Neurology
|October 22, 2008
Summary
This study analyzed primary CNS lymphoma (PCNSL) with eye involvement in 221 patients. Dedicated ocular therapy improved disease control but did not impact overall survival rates.
Area of Science:
- Neuro-oncology
- Ophthalmology
- Hematology
Background:
- Primary CNS lymphoma (PCNSL) is a rare malignancy.
- Ocular involvement in PCNSL presents unique diagnostic and therapeutic challenges.
- Understanding the characteristics of PCNSL with intraocular involvement is crucial for patient management.
Purpose of the Study:
- To detail the demographics, diagnosis, treatment, and outcomes of patients with PCNSL and ocular involvement.
- To evaluate the impact of dedicated ocular therapy on disease control and survival.
- To contribute to the largest reported series of PCNSL with intraocular involvement.
Main Methods:
- Retrospective analysis of 221 HIV-negative, immunocompetent patients with brain and ocular lymphoma from 16 international centers.
- Data collected on demographics, presenting symptoms, diagnostic methods (brain biopsy, vitrectomy, CSF cytology), and treatment strategies.
- Evaluation of treatment response, progression sites, progression-free survival (PFS), and overall survival (OS).
Main Results:
- Median age at diagnosis was 60 years, with 57% women. Ocular disturbance and cognitive changes were common symptoms.
- Diagnosis involved brain biopsy (147), vitrectomy (65), or CSF cytology (11). Intraocular lymphoma diagnosed via biopsy (90) or ophthalmic exam (141).
- Median PFS and OS were 18 and 31 months, respectively. Dedicated ocular therapy improved disease control but not overall survival.
Conclusions:
- This study represents the largest cohort of PCNSL patients with intraocular involvement.
- Survival outcomes for PCNSL with ocular involvement are comparable to those without ocular disease.
- While local ocular therapies enhance disease control, they do not significantly alter the overall survival of patients with PCNSL.
