Primary central nervous system lymphoma—current standards and recent developments

Neuro-Oncology Practice, vol. 13, pp. 640–648

Abstract

Primary central nervous system lymphoma (PCNSL) is a rare aggressive B-cell non-Hodgkin lymphoma confined to the central nervous system, without systemic involvement. The incidence has increased over the past 3 decades. The prognosis has improved in patients up to 70 years old, and this type of lymphoma can be potentially cured. The gold standard of diagnosing PCNSL is histological, usually on a brain biopsy specimen via a neurosurgical procedure. Recent developments in both imaging and laboratory analyses of the cerebrospinal fluid, can be helpful in narrowing the differential diagnosis, diagnosing PCNSL itself, and in follow-up after treatment. This narrative review gives an overview of the epidemiology, diagnosis, and treatment of PCNSL, with an emphasis on recent developments in diagnostic techniques and treatment.

Authors 7

1. Matthijs van der Meulen corresponding Aachen Department of Neurology, Leiden University Medical Center (LUMC)

   Leiden University Medical Center

   Affiliation as printed

   Department of Neurology, Leiden University Medical Center , Leiden,

2. Joost S.P. Vermaat Aachen Department of Hematology, Leiden University Medical Center

   Leiden University Medical Center

   Affiliation as printed

   Department of Hematology, Leiden University Medical Center , Leiden,

3. Norbert Galldiks

   Forschungszentrum Jülich · University Hospital Cologne

   Affiliation as printed

   Department of Neurology, University Hospital Cologne , Cologne,

   Institute of Neuroscience and Medicine (INM-3), Research Center Juelich , Juelich,

4. Samar Issa

   Middlemore Hospital

   Affiliation as printed

   Department of Hematology, Middlemore Hospital , Auckland,

5. Jeroen de Bresser Aachen Department of Radiology, Leiden University Medical Center

   Leiden University Medical Center

   Affiliation as printed

   Department of Radiology, Leiden University Medical Center , Leiden,

6. Jeanette K. Doorduijn

   Erasmus MC · Erasmus MC Cancer Institute

   Affiliation as printed

   Department of hematology, Erasmus MC Cancer Institute, University Medical Center Rotterdam , Rotterdam,

7. Jacoline E. C. Bromberg

   Erasmus MC · Erasmus MC Cancer Institute

   Affiliation as printed

   Department of neurology, Erasmus MC Cancer Institute, Brain Tumor Center, University Medical Center Rotterdam , Rotterdam,