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Prognostic Scoring System for Primary CNS Lymphomas: The International Extranodal Lymphoma Study Group Experience

Andrés J.M. Ferreri, Jean-Yves Blay, Michele Reni, Felice Pasini, Michele Spina, Achille Ambrosetti, Antonello Calderoni, Andrea Rossi, Vittorio Vavassori, Annarita Conconi, Liliana Devizzi, Françoise Berger, Maurilio Ponzoni, Bettina Borisch, Marianne Tinguely, Michele Cerati, Mario Milani, Enrico Orvieto, Juvenal Sanchez, Christine Chevreau, Stefania Dell’Oro, Emanuele Zucca, Franco Cavalli

From the Departments of Radiochemotherapy and Pathology, San Raffaele H Scientific Institute, and Divisione di Oncologia Medica, Istituto Nazionale dei Tumori, Milan; Divisione di Radioterapia, Ospedale di Circolo e Fondazione Macchi, and Anatomia e Istologia Patologica, Ospedale di Circolo Fondazione Macchi, Università dell’Insubria, Varese; Divisione Clinicizzata di Oncologia Medica, Ospedale Civile Maggiore, and Cattedra di Ematologia, Università di Verona, Ospedale Policlinico di Borgo Roma, Verona; Division of Medical Oncology "A," Centro di Riferimento Oncologico, National Cancer Institute, Aviano; Divisione di Ematologia, Ospedali Riuniti di Bergamo, Bergamo; Divisione di Anatomia Patologica, Azienda Ospedaliera di Lecco, Lecco; Istituto di Anatomia e Istologia Patologica, Ospedale Regionale di Treviso, Treviso, Italy; Unité Cytokines et Cancers, Hopital E. Herriot & Centre Léon Bérard, and Service de Pathologie, Centre Hospitalier Lyon-Sud, Lyon; Centre Claudius Regaud, Toulouse, France; Istituto Oncologico della Svizzera Italiana, Ospedale San Giovannia, Bellinzona; Institut für Medizinische Onkologie Inselspital, Bern; Division de Pathologie Clinique, Hôpitaux Universitaires de Genève, Hôpital Cantonal, Genève, Switzerland; and Departamento de Anatomia Patológica, Instituto de Enfermedades Neoplásicas, Lima, Perú.

Address reprint requests to Andrés J.M. Ferreri, MD, Department of Radiochemotherapy, San Raffaele H Scientific Institute, via Olgettina 60, 20132, Milan, Italy; email: andres.ferreri{at}hsr.it.

Purpose: To identify survival predictors and to design a prognostic score useful for distinguishing risk groups in immunocompetent patients with primary CNS lymphomas (PCNSL).

Patients and Methods: The prognostic role of patient-, lymphoma-, and treatment-related variables was analyzed in a multicenter series of 378 PCNSL patients treated at 23 cancer centers from five different countries.

Results: Age more than 60 years, performance status (PS) more than 1, elevated lactate dehydrogenase (LDH) serum level, high CSF protein concentration, and involvement of deep regions of the brain (periventricular regions, basal ganglia, brainstem, and/or cerebellum) were significantly and independently associated with a worse survival. These five variables were used to design a prognostic score. Each variable was assigned a value of either 0, if favorable, or 1, if unfavorable. The values were then added together to arrive at a final score, which was tested in 105 assessable patients for which complete data of all five variables were available. The 2-year overall survival (OS) ± SD was 80% ± 8%, 48% ± 7%, and 15% ± 7% (P = .00001) for patients with zero to one, two to three, and four to five unfavorable features, respectively. The prognostic role of this score was confirmed by limiting analysis to assessable patients treated with high-dose methotrexate-based chemotherapy (2-year OS ± SD: 85% ± 8%, 57% ± 8%, and 24% ± 11%; P = .0004).

Conclusion: Age, PS, LDH serum level, CSF protein concentration, and involvement of deep structures of the brain were independent predictors of survival. A prognostic score including these five parameters seems advisable in distinguishing different risk groups in PCNSL patients. The proposed score and its relevance in therapeutic decision deserve to be validated in further studies.

Presented at the Forty-Second Annual Meeting of the American Society of Hematology, December 6–10, San Francisco, CA, 2000

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