Primary CNS lymphomas (PCNSLs) are defined as non-Hodgkin lymphomas which at the time of
diagnosis are located exclusively in the brain parenchyma in the meninges and or in the
spinal cord or in the vitreoretinal area. They occur much more frequently in immunoincompetent
patients than in immunocompetent patients. As a rule it is an aggressive diffuse large-cell
B-cell lymphoma in which the median survival time without therapy is only a few weeks to
months after diagnosis.The therapeutic spectrum of PCNSL is limited by the difficulty of
crossing the blood-brain barrier. If this is the case however the chemosensitivity is high.
Due to the rarity of this tumor disease most studies are based only on small numbers of
patients with limited informative value. Nevertheless in the last few years it has been
possible to establish promising curative therapeutic approaches that are based on induction and
subsequent consolidation therapy and enable high remission rates and several years of
progression-free survival.All of these aspects are taken into account in this monograph which
summarizes the current knowledge on the PCNSLs in a compact and informative way.
