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Annals of Oncology Advance Access originally published online on September 5, 2007
Annals of Oncology 2007 18(11):1851-1855; doi:10.1093/annonc/mdm340
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© 2007 European Society for Medical Oncology

CNS tumors

Primary intraocular lymphoma: an International Primary Central Nervous System Lymphoma Collaborative Group Report

S. A. Grimm1, J. S. Pulido2, K. Jahnke3, D. Schiff4, A. J. Hall5, T. N. Shenkier6, T. Siegal7, N. D. Doolittle8, T. Batchelor9, U. Herrlinger10, E. A. Neuwelt8,11, N. Laperriere12, M. C. Chamberlain13, J. Y. Blay14, A. J. M. Ferreri14, A. M. P. Omuro15, E. Thiel3 and L. E. Abrey1,*

1 Memorial Sloan-Kettering Cancer Center, New York
2 Mayo Clinic, Rochester
3 Charité University Medicine Berlin, Campus Benjamin Franklin, Berlin, Germany
4 University of Virginia, Charlottesville
5 Royal Melbourne Hospital and Royal Victorian Eye and Ear Hospital, Melbourne, Australia
6 British Columbia Cancer Agency, Vancouver, Canada
7 Hadassah Hebrew University Hospital, Jerusalem, Israel
8 Oregon Health & Sciences University, Portland
9 Massachusetts General Hospital, Boston
10 University of Tuebingen, Tuebingen, Germany
11 Portland VA Medical Center, Portland
12 Princess Margaret Hospital, Toronto, Canada
13 Moffitt Cancer Center, Tampa
14 On behalf of the International Extranodal Lymphoma Study Group
15 Hospital Pitie-Salpetriere, Paris, France

* Correspondence to: Dr L. E. Abrey, Department of Neurology, Memorial Sloan-Kettering Cancer Center, 1275 York Avenue, New York, NY 10021, USA. Tel: +1-212-639-5122; Fax: +1-917-432-2310; E-mail: abreyl{at}mskcc.org

Background: Primary intraocular lymphoma (PIOL) is an uncommon subset of primary central nervous system lymphoma. Because it is rare and difficult to diagnose, the natural history and optimal management are unknown.

Patients and methods: A retrospective study of 83 HIV negative, immunocompetent PIOL patients was assembled from 16 centers in seven countries.

Results: Median age at diagnosis was 65. Median ECOG performance status was 0. Presenting symptoms included blurred vision, decreased visual acuity, and floaters. Median time to diagnosis was 6 months. Diagnosis was made by vitrectomy (74), choroidal/retinal biopsy (6) and ophthalmic exam (3). Eleven percent had positive CSF cytology. Initial treatment was categorized as focal in 23 (intra-ocular methotrexate, ocular radiotherapy) or extensive in 53 (systemic chemotherapy, whole brain radiotherapy). Six received none; details are unknown in one. Forty-seven relapsed: brain 47%, eyes 30%, brain and eyes 15%, and systemic 8%. Median time to relapse was 19 months. Focal therapy alone did not increase risk of brain relapse. Median progression free (PFS) and overall survival (OS) were 29.6 and 58 months, respectively, and unaffected by treatment type.

Conclusion: Treatment type did not affect relapse pattern, median PFS or OS. Focal therapy may minimize treatment toxicity without compromising disease control.

Key words: Ocular lymphoma, primary CNS lymphoma

Received for publication May 25, 2007. Accepted for publication May 30, 2007.


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S. A. Grimm, C. A. McCannel, A.M.P. Omuro, A. J.M. Ferreri, J. -Y. Blay, E. A. Neuwelt, T. Siegal, T. Batchelor, K. Jahnke, T. N. Shenkier, et al.
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