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NEUROLOGY 2008;71:1355-1360
© 2008 American Academy of Neurology

Primary CNS lymphoma with intraocular involvement

International PCNSL Collaborative Group Report

S. A. Grimm, MD, C. A. McCannel, MD, A.M.P. Omuro, MD, A. J.M. Ferreri, MD, J. -Y. Blay, MD, E. A. Neuwelt, MD, T. Siegal, MD, T. Batchelor, MD, K. Jahnke, MD, T. N. Shenkier, MD, A. J. Hall, MD, F. Graus, MD, U. Herrlinger, MD, D. Schiff, MD, J. Raizer, MD, J. Rubenstein, MD, N. Laperriere, MD, E. Thiel, MD, N. Doolittle, PhD, F. M. Iwamoto, MD and L. E. Abrey, MD

From Memorial Sloan-Kettering Cancer Center (S.A.G., F.M.I., L.E.A.), New York, NY; Mayo Clinic (C.A.M.), Rochester, MN; Hospital Pitie-Salpetriere (A.M.P.O.), Paris, France; International Extranodal Lymphoma Study Group (A.J.M.F.. J.-Y.B.); Oregon Health & Sciences University (E.A.N., N.D.), Portland; Hadassah Hebrew University Hospital (T.S.), Jerusalem, Israel; Massachusetts General Hospital (T.B.), Boston; Charité University Medicine-Berlin (K.J., E.T.), Campus Benjamin Franklin, Berlin, Germany; British Columbia Cancer Agency (T.N.S.), Vancouver, Canada; Royal Melbourne Hospital and Royal Victorian Eye and Ear Hospital (A.J.H.), Melbourne, Australia; Hospital Clinic (F.G.), Institut d’Investigacio Biomedica August Pi i Sunyer, Barcelona, Spain; University of Tuebingen (U.H.), Germany; University of Virginia (D.S.), Charlottesville; Northwestern Memorial Hospital (J.R.), Chicago, IL; University of California at San Francisco (J.R.); and Princess Margaret Hospital (N.L.), Toronto, Canada.

Address correspondence and reprint requests to Dr. Lauren E. Abrey, Department of Neurology, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10021 abreyl{at}mskcc.org

Objective: To describe the demographics, diagnostic details, therapeutic management, and outcome in patients with primary CNS lymphoma (PCNSL) with ocular involvement.

Methods: A retrospective study of 221 patients was assembled from 16 centers in seven countries. Only HIV-negative, immunocompetent patients with brain and ocular lymphoma were included; none had systemic lymphoma.

Results: Median age at diagnosis was 60. Fifty-seven percent were women. Median Eastern Cooperative Oncology Group performance status was 2. Ocular disturbance and behavioral/cognitive changes were the most common presenting symptoms. Diagnosis of lymphoma was made by brain biopsy (147), vitrectomy (65), or CSF cytology (11). Diagnosis of intraocular lymphoma was made by vitrectomy/choroidal/retinal biopsy (90) or clinical ophthalmic examination (141). CSF cytology was positive in 23%. Treatment information was available for 176 patients. A total of 102 received dedicated ocular therapy (ocular radiotherapy 79, intravitreal methotrexate 22, and both 1) in addition to treatment for their brain lymphoma. Sixty-nine percent progressed at a median of 13 months; sites of progression included brain 52%, eyes 19%, brain and eyes 12%, and systemic 2%. Patients treated with local ocular therapy did not have a statistically significant decreased risk of failing in the eyes (p = 0.7). Median progression free survival and overall survival for the entire cohort were 18 and 31 months.

Conclusion: This is the largest reported series of primary CNS lymphoma (PCNSL) with intraocular involvement. Progression free and overall survival was similar to that reported with PCNSL. Dedicated ocular therapy improved disease control but did not affect overall survival.

Abbreviations: ECOG = Eastern Cooperative Oncology Group; IPCG = International Primary Central Nervous System Lymphoma Collaborative Group; IT = intrathecal chemotherapy; MTX = methotrexate; PCNSL = primary CNS lymphoma; PFS = progression-free survival; RT = radiotherapy; WBRT = whole brain radiotherapy.

Disclosure: The authors report no disclosures.

Presented in part at the Society for Neuro-Oncology, 2006, and the American Academy of Neurology, 2007.

Received March 26, 2008. Accepted in final form July 15, 2008.






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