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Annals of Oncology 13:531-538, 2002
© 2002 European Society for Medical Oncology

Original Paper

Relevance of intraocular involvement in the management of primary central nervous system lymphomas

A. J. M. Ferreri1,+, J.-Y. Blay2, M. Reni3, F. Pasini4, A. Gubkin5, U. Tirelli6, A. Calderoni7, E. Zucca8, S. Cortelazzo9, C. Chassagne10, M. Tinguely11, B. Borisch11, F. Berger12, M. Ponzoni13 and F. Cavalli8,§

1Department of Radiochemotherapy, San Raffaele H Scientific Institute, Milan, Italy; 2Unité Cytokines et Cancers, Hopital E. Herriot et Centre Léon Bérard, Lyon, France; 3Divisione di Radioterapia, Ospedale di Circolo Fondazione Macchi, Varese, Italy; 4Divisione Clinicizzata di Oncologia Medica, Ospedale Civile Maggiore, Verona, Italy; 5Haematology and Intensive Care Department, Haematological Centre of Russian Academy of Medical Sciences, Moscow, Russia; 6Divisione of Medical Oncology A, Centro di Riferimento Oncologico, National Cancer Institute, Aviano, Italy; 7Institut für Medizinische Onkologie Inselspital, Bern, Switzerland; 8Istituto Oncologico della Svizzera Italiana, Ospedale San Giovanni, Bellinzona, Switzerland; 9Divisione di Ematologia, Ospedale Riuniti di Bergamo, Bergamo, Italy; 10Service de Pathologie, Centre Léon Bérard, Lyon, France; 11Divisione de Pathologie Clinique CMU, Hôpitaux Universitaires de Genève, Hôpital Cantonal, Genève, Switzerland; 12Service de Pathologie, Centre Hospitalier Lyon-Sud, Pierre Bénite, France; 13Department of Pathology, San Raffaele H Scientific Institute, Milan, Italy

Received 25 July 2002; revised 21 September 2001; accepted 5 October 2001.

Background

Reported data regarding intraocular lymphoma (IOL) management are anecdotal. Cases of IOL included in an international multicentre series of 378 immunocompetent patients with primary central nervous system lymphomas (PCNSLs) were reviewed.

Patients and methods

Staging included slit-lamp examination in 170 patients: IOL was diagnosed in 22 cases (13%). A concomitant brain lesion was detected in 21 cases. Planned treatment was chemotherapy followed by radiotherapy in 13 cases, chemotherapy alone in three and radiotherapy, followed by or not by chemotherapy in five; one patient was not treated. Chemotherapy included high-dose methotrexate in 12 cases. Ten patients received intrathecal chemotherapy. Radiotherapy consisted of whole brain irradiation, followed by or not by a tumour bed boost; ocular irradiation was planned in 15 cases. Irradiation in one patient without brain lesions was limited to the orbits only (50 Gy).

Results

IOL was positively correlated to systemic symptoms and meningeal disease. Fifteen patients (71%) achieved an objective response; 16 patients experienced a failure (2-year failure-free survival 34 ± 10%). Failures involved the eyes in eight cases, with a 2-year time to ocular relapse of 59 ± 11%. Ocular failure was less common in patients treated with chemotherapy plus ocular irradiation and was associated with a significantly shorter survival. Seven patients are alive [median follow-up 53 months, 2-year overall survival (OS): 39 ± 11%], five of whom were treated with ocular irradiation. The patient with isolated IOL is alive and disease-free at 14 months. OS of the entire series was similar to that of PCNSL patients with negative slit-lamp examination.

Conclusions

IOL is usually associated with concomitant brain disease and shows a survival similar to that of the rest of PCNSLs. Chemotherapy combined with ocular irradiation resulted in better control of ocular disease, which seems to be associated with survival. In view of the potential role of ocular irradiation, the use of chemotherapy alone in phase II trials should be critically reconsidered in PCNSL patients with ocular disease.

Key words: chemotherapy, combined treatment, eye, intraocular lymphoma, ocular irradiation, primary central nervous system lymphoma


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