Primary Central Nervous System Lymphoma: Systemic Relapse

  • Hervé Ghesquières
Part of the Tumors of the Central Nervous System book series (TCNS, volume 9)


Primary CNS lymphoma (PCNSL) is characterized by the presence of lymphoma cells limited to the CNS, with possible involvement of the eyes or the cerebrospinal fluid. A majority of PCNSL are diffuse large B-cell lymphomas with an aggressive course. Retrospective series show that about 4% of patients (range, 0–24%) may experience a systemic relapse, mainly with unusual extra-nodal involvement. Systemic relapse can occur very early after initial diagnosis of PCNSL, raising the question of the existence of a systemic lymphoma going undetected at the time of conventional PCNSL staging. Late relapse can occur more than 5 years after the diagnosis, suggesting the development of a second lymphoma or the emergence of a systemic lymphoma arising from circulating clones. Globally, the prognosis is poor but seems slightly more favorable than for patients with intra-cerebral relapse. The present chapter reviews the biological implications of systemic relapse in the pathogenesis of PCNSL.


Median Overall Survival Autologous Stem Cell Transplantation Primary Central Nervous System Lymphoma Central Nervous System Relapse Systemic Relapse 
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Copyright information

© Springer Science+Business Media Dordrecht 2012

Authors and Affiliations

  1. 1.Department of Medical Oncology, Centre Léon BérardUniversité de LyonLyonFrance

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