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Neuro-Oncology

Primary CNS Lymphoma in Immunocompetent Patients

^aAP-HP, Groupe Hospitalier Pitié-Salpêtrière, Service de Neurologie Mazarin, Paris, France; ^bAP-HP, Groupe Hospitalier Pitié-Salpêtrière, Laboratoire de Neuropathologie R Escourolle, ^cINSERM, Paris, France; ^dCentre René-Huguenin, Service d'Hématologie, Saint-Cloud, France; ^eHôpital du Val de Grâce, Service de Neurologie, Paris, France; ^fUniversité Pierre et Marie Curie-Paris 6, UPMC, Laboratoire Biologie des Interactions Neurone-Glie, Groupe Hospitalier Pitié-Salpêtrière, Paris, France

Key Words. Primary CNS lymphoma • Molecular genetics • Chemotherapy • Stem cell transplantation • Rituximab • Neurotoxicity

Correspondence: Khê Hoang-Xuan, M.D., Ph.D., Service de Neurologie Mazarin, Groupe Hospitalier Pitié-Salpêtrière, Université Pierre et Marie Curie (UPMC), 47 Boulevard de l'Hôpital, Paris 75651 Cedex 13, France. Telephone: 33-1-42160573; Fax: 33-1-42160375; e-mail: khe.hoang-xuan{at}psl.aphp.fr

Received November 3, 2008; accepted for publication April 20, 2009; first published online in THE ONCOLOGIST Express on May 11, 2009.

Disclosures: Monica Sierra del Rio: None; Audrey Rousseau: None; Carole Soussain: None; Damien Richard: None; Khê Hoang-Xuan: None.

The content of this article has been reviewed by independent peer reviewers to ensure that it is balanced, objective, and free from commercial bias. No financial relationships relevant to the content of this article have been disclosed by the authors or independent peer reviewers.

Primary central nervous system lymphoma (PCNSL) constitutes a rare group of extranodal non-Hodgkin's lymphomas (NHLs), primarily of B cell origin, whose incidence has markedly increased in the last three decades. Immunodeficiency is the main risk factor, but the large majority of patients are immunocompetent. Recent evidence suggests a specific tumorigenesis that may explain their particular clinical behavior compared with systemic NHL. The addition of i.v. high-dose methotrexate (MTX) chemotherapy to whole-brain radiotherapy (WBRT) has considerably improved the prognosis, leading to a threefold longer median survival time compared with WBRT alone and represents the current standard of care. However, this combined treatment exposes the patient, especially the elderly, to a high risk for delayed neurotoxicity. In the older population (>60 years), there is growing evidence that MTX-based chemotherapy alone as initial treatment is the best approach to achieve effective tumor control without compromising patient quality of life. In the younger population, the risk for neurotoxicity is much lower, and this strategy is controversial because it may be associated with higher relapse rates. Future efforts should focus on the development of new polychemotherapy regimens allowing the reduction or deferral of WBRT in order to minimize the risk for delayed neurotoxicity. In this setting, intensive chemotherapy with autologous blood stem cell transplantation was recently demonstrated to be feasible and efficient as salvage therapy and is currently being evaluated as part of primary treatment. This review highlights the recent advances in the pathogenesis and treatment of PCNSL in the immunocompetent population.